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Aryal S, Zhu‐qian J, Qiang LY, Haque MA. Mimicry unveiled: The challenging diagnosis of pigmented purpura-like mycosis fungoides initially misdiagnosed as pigmented purpura. Clin Case Rep 2024; 12:e8847. [PMID: 38770416 PMCID: PMC11103549 DOI: 10.1002/ccr3.8847] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2024] [Revised: 03/29/2024] [Accepted: 04/15/2024] [Indexed: 05/22/2024] Open
Abstract
Key Clinical Message Unlike most cases, the lesions were localized to the dorsum of the hand, lacked pruritus (itching), and did not exhibit "sperm-like blood vessels," which are typically pathognomonic to classical MF. Abstract The study presents a rare case involving a 44-year-old woman who developed a skin condition on the base of her left thumb. Initially misdiagnosed as pigmented purpura, the need for further investigation arose to determine the nature of the condition accurately. The medical evaluation encompassed a comprehensive analysis of the patient's skin ailment. A series of diagnostic examinations were conducted to ascertain the underlying cause. Although routine blood tests yielded unremarkable results, the distinct characteristics of the rash prompted a more thorough investigation. Subsequent assessment revealed that the skin condition was not pigmented purpura, as initially presumed, but rather a manifestation of cutaneous T-cell lymphoma (CTCL) known as mycosis fungoides (MF). MF is an infrequent lymphoma predominantly affecting individuals aged 45-65, exhibiting a male-to-female sex ratio of 2:1. The annual incidence of MF ranges from 0.3 to 0.96 cases per 100,000 individuals. The woman's skin exhibited discrete patches adorned with colored dots, progressively thickening and pigmentation. Notably, the absence of pruritus did not dispel suspicion. This case underscores the significance of accurately diagnosing uncommon dermatological disorders to facilitate appropriate medical intervention. The unique appearance of the rash and its distinctive features, despite normal blood results, enabled the identification of MF. The patient's treatment encompassed a combination of steroids and narrowband UV therapy. Vigilance, continued research, and heightened awareness are paramount for early intervention and improved patient outcomes. Such efforts contribute to an enhanced understanding of the complexities of this condition.
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Affiliation(s)
- Sabita Aryal
- Department of Dermatology, Shanghai Skin Diseases HospitalTongji UniversityShanghaiChina
- Shanghai Skin Diseases Hospital, School of MedicineTongji UniversityShanghaiChina
| | - Jiang Zhu‐qian
- Department of DermatologyShanghai Jiading Hospital of Traditional Chinese MedicineShanghaiChina
| | - Liu ye Qiang
- Department of Dermatology, Shanghai Skin Diseases HospitalTongji UniversityShanghaiChina
- Shanghai Skin Diseases Hospital, School of MedicineTongji UniversityShanghaiChina
| | - Md Ariful Haque
- Department of Orthopaedic SurgeryYan'an Hospital Affiliated to Kunming Medical UniversityKunmingYunnanChina
- Department of Public HealthAtish Dipankar University of Science and TechnologyDhakaBangladesh
- Voice of Doctors Research SchoolDhakaBangladesh
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2
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Oska S, Barash A, D'Sa H, Pui J, Hristov A, Sundram UN. Verrucous Plaques in a Young Woman: Answer. Am J Dermatopathol 2024; 46:132. [PMID: 38275241 DOI: 10.1097/dad.0000000000002609] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/27/2024]
Affiliation(s)
- Sandra Oska
- Department of Dermatology, Henry Ford Hospital, Detroit, MI
| | | | - Helen D'Sa
- Middlebelt Dermatology, Farmington Hills, MI
| | - John Pui
- Department of Anatomic Pathology, Corewell Health East, Farmington Hills, MI
| | - Alexandra Hristov
- Department of Pathology
- Department of Dermatology, Michigan Medicine, Ann Arbor, MI; and
| | - Uma N Sundram
- Department of Anatomic Pathology, Corewell Health East, Royal Oak, MI
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3
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Zhao P, Gish TJ, Mannschreck D, Marchi E, Cropley TG. Zosteriform mycosis fungoides and lymphomatoid papulosis arising in an area of prior herpes zoster. JAAD Case Rep 2023; 40:84-88. [PMID: 37771361 PMCID: PMC10523180 DOI: 10.1016/j.jdcr.2023.08.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/30/2023] Open
Affiliation(s)
- Patricia Zhao
- University of Virginia School of Medicine, Charlottesville, Virginia
| | - Tappy J. Gish
- Department of Pathology, University of Virginia Medical Center, Charlottesville, Virginia
| | - Diana Mannschreck
- Department of Dermatology, University of Virginia Medical Center, Charlottesville, Virginia
| | - Enrica Marchi
- Division of Hematology & Oncology, Department of Medicine, University of Virginia Medical Center, Charlottesville, Virginia
| | - Thomas G. Cropley
- Department of Dermatology, University of Virginia Medical Center, Charlottesville, Virginia
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4
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Ozcan Y, Ozlu E, Karagun E, Uyar B, Gamsizkan M. Dermatopathological Correlation of Clinically Challenging Cutaneous Lesions: a Single Center Experience of 2184 Cases. Dermatol Pract Concept 2022; 12:e2022186. [PMID: 36534561 PMCID: PMC9682974 DOI: 10.5826/dpc.1204a186] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/07/2022] [Indexed: 01/25/2023] Open
Abstract
INTRODUCTION Although a trained eye can easily identify typical skin lesions, histopathological examination and clinicopathological correlation are critical in challenging cases. OBJECTIVES The primary objective is to organize the final diagnoses reached following clinicopathological consensus in clinically challenging cutaneous lesions, identifying the most common diagnostic scenarios encountered by dermatopathologists and discussing their diverse differentials submitted by clinicians. The secondary objective is to investigate how the case profile and clinician decision-making processes evolved during the COVID-19 pandemic. METHODS Skin and mucosa samples collected by the dermatology department between 2016 and 2020 were classified based on pathology reports. For frequent diagnoses, preliminary diagnoses stated by clinicians on pathology requisition forms were reviewed. The years preceding and following the first nationally reported COVID-19 case were compared to investigate the pandemic's impact on the distribution of dermatology and dermatopathology cases. RESULTS One thousand nine hundred and eighty-nine reports were classified into 4 major categories: inflammatory (49.8%), neoplastic (30.1%), other diseases (7.1%), and non-diagnostic (12.8%). We further classified inflammatory diseases based on major tissue reaction patterns and neoplasms based on cell origin. We analyzed the leading diagnoses in each category, discussed their differential diagnoses, and provided clinicians with clues to reduce errors in practice. Following the pandemic, the overall number of pathology reports and patient admissions dropped dramatically, with significant changes in case profiles. CONCLUSIONS We presented and discussed the frequently encountered confounding cases to sketch the diagnostic landscape. In the authors' experience, clinicopathological correlation can increase the rate of reaching the diagnosis by up to 75.3%.
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Affiliation(s)
- Yunus Ozcan
- Department of Dermatology, Duzce Ataturk State Hospital, Duzce, Turkey
| | - Emin Ozlu
- Department of Dermatology, Faculty of Medicine, Duzce University, Duzce, Turkey
| | - Ebru Karagun
- Department of Dermatology, Faculty of Medicine, Istinye University, Istanbul, Turkey
| | - Belkiz Uyar
- Department of Dermatology, Faculty of Medicine, Duzce University, Duzce, Turkey
| | - Mehmet Gamsizkan
- Department of Pathology, Faculty of Medicine, Duzce University, Duzce, Turkey
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5
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Khalil S, Donthi D, Gru AA. Cutaneous Reactive B-cell Lymphoid Proliferations. J Cutan Pathol 2022; 49:898-916. [PMID: 35656820 DOI: 10.1111/cup.14264] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2021] [Revised: 05/14/2022] [Accepted: 05/23/2022] [Indexed: 11/28/2022]
Abstract
Cutaneous lymphoid hyperplasia (CLH), also known as cutaneous pseudolymphoma, is a spectrum of benign conditions characterized by reactive B- and T-cell cutaneous lymphocytic infiltrates. B-cell lymphoid proliferations are a heterogenous group of non-neoplastic cutaneous diseases that must be histopathologically distinguished from cutaneous B-cell lymphomas. These proliferations can be observed as reactive phenomena to infections, medications, allergens, neoplasms, and more. Further, there are many inflammatory conditions that present with reactive B-cell infiltrates, including actinic prurigo, Zoon balanitis, Rosai-Dorfman, and cutaneous plasmacytosis. This review summarizes multiple cutaneous B-cell lymphoid proliferations within the major categories of reactive and disease-associated CLH. Further we discuss major discriminating features of atypical CLH and malignancy. Understanding the specific patterns of B-cell CLH is essential for the proper diagnosis and treatment of patients presenting with such lesions.
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Affiliation(s)
- Shadi Khalil
- Department of Dermatology, University of California San Diego
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6
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Uysal Pİ, Ayva EŞ, Tepeoğlu M, Uysal AC. Solitary purpuric plaque in a four-year-old girl: Histopathological diagnostic challenge. Indian J Dermatol Venereol Leprol 2022; 88:541-543. [PMID: 35593288 DOI: 10.25259/ijdvl_1039_2021] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2021] [Accepted: 01/01/2022] [Indexed: 11/04/2022]
Affiliation(s)
- Pinar İncel Uysal
- Department of Dermatology, TOBB ETU Faculty of Medicine, Yasam cad. No. 5, Baskent University, Ankara, Turkey
| | - Ebru Şebnem Ayva
- Department of Pathology, Reconstructive and Aesthetic Surgery, Baskent University Faculty of Medicine, Baskent University, Ankara, Turkey
| | - Merih Tepeoğlu
- Department of Pathology, Reconstructive and Aesthetic Surgery, Baskent University Faculty of Medicine, Baskent University, Ankara, Turkey
| | - Ahmet Cagri Uysal
- Department of Plastic, Reconstructive and Aesthetic Surgery, Baskent University Faculty of Medicine, Baskent University, Ankara, Turkey
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7
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Nasimi M, Bonabiyan M, Lajevardi V, Azizpour A, Nejat A, Dasdar S, Kianfar N. Pigmented purpuric dermatoses versus purpuric mycosis fungoides: Clinicopathologic similarities and new insights into dermoscopic features. Australas J Dermatol 2021; 63:81-85. [PMID: 34905635 DOI: 10.1111/ajd.13759] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2021] [Revised: 11/03/2021] [Accepted: 11/21/2021] [Indexed: 11/28/2022]
Abstract
BACKGROUND The diagnosis of purpuric mycosis fungoides (PMF) is often challenging to be clinically differentiated from inflammatory diseases such as pigmented purpuric dermatosis (PPD). Dermoscopy as a non-invasive method can be employed for the visualisation of features invisible to the naked eye. OBJECTIVES This study aimed to survey the dermoscopic findings of PMF in comparison with PPD. METHODS Forty-one patients with an established diagnosis of PMF (n = 28) and PPD (n = 13) were prospectively recruited. Dermoscopic images were taken by FotoFinder Medicam 1000 (FotoFinder Systems GmbH, Bad Birnbach, Germany). RESULTS Characteristic dermoscopic patterns consisting of fine short linear vessels (35.7%, P = 0.017) and spermatozoa-like structures (50%, P = 0.014) were found to be significantly more common in PMF lesions, while PPD lesions were typified by erythematous globules (76.9%, P = 0.01), in the background colour of dull red (61.5%, P = 0.01) and reticular pigmentation (61.5%, P = 0.044). CONCLUSIONS This study showed the usefulness of dermoscopy for the diagnosis of PMF and PPD cases. Studies with long-term follow-up are needed to affirm the value of these dermoscopic patterns in the differentiation between the two entities.
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Affiliation(s)
- Maryam Nasimi
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Masoud Bonabiyan
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Vahideh Lajevardi
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Arghavan Azizpour
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Afsaneh Nejat
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Shayan Dasdar
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Nika Kianfar
- Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
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8
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Liu Z, Wu X, Hwang ST, Liu J. The Role of Tumor Microenvironment in Mycosis Fungoides and Sézary Syndrome. Ann Dermatol 2021; 33:487-496. [PMID: 34858000 PMCID: PMC8577908 DOI: 10.5021/ad.2021.33.6.487] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2020] [Revised: 03/21/2021] [Accepted: 04/06/2021] [Indexed: 11/25/2022] Open
Abstract
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphomas (CTCLs). Most cases of MF display an indolent course during its early stage. However, in some patients, it can progress to the tumor stage with potential systematic involvement and a poor prognosis. SS is defined as an erythrodermic CTCL with leukemic involvements. The pathogenesis of MF and SS is still not fully understood, but recent data have found that the development of MF and SS is related to genetic alterations and possibly to environmental influences. In CTCL, many components interacting with tumor cells, such as tumor-associated macrophages, fibroblasts, dendritic cells, mast cells, and myeloid-derived suppressor cells, as well as with chemokines, cytokines and other key players, establish the tumor microenvironment (TME). In turn, the TME regulates tumor cell migration and proliferation directly and indirectly and may play a critical role in the progression of MF and SS. The TME of MF and SS appear to show features of a Th2 phenotype, thus dampening tumor-related immune responses. Recently, several studies have been published on the immunological characteristics of MF and SS, but a full understanding of the CTCL-related TME remains to be determined. This review focuses on the role of the TME in MF and SS, aiming to further demonstrate the pathogenesis of the disease and to provide new ideas for potential treatments targeted at the microenvironment components of the tumor.
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Affiliation(s)
- Zhaorui Liu
- Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
| | - Xuesong Wu
- Department of Dermatology, School of Medicine, University of California Davis, Sacramento, CA, United States
| | - Sam T Hwang
- Department of Dermatology, School of Medicine, University of California Davis, Sacramento, CA, United States
| | - Jie Liu
- Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
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9
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Kolm I, Buset C, Flury U, Nosek D, Kazakov DV, Kempf W. Lichen aureus with pseudolymphomatous infiltrate. J Cutan Pathol 2021; 48:669-673. [PMID: 33368548 DOI: 10.1111/cup.13948] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Revised: 12/14/2020] [Accepted: 12/21/2020] [Indexed: 12/16/2022]
Abstract
Lichen aureus is a variant of pigmented purpuric dermatoses. The usual histopathology of lichen aureus is characterized by a subepidermal dense, band-like lymphocytic infiltrate, extravasated erythrocytes, and hemosiderin deposits. We report three patients with lichen aureus on the extremities with similar clinical, dermoscopic, and histopathological findings characterized by a dense band-like relatively deep dermal infiltrate accompanied by extravasation of erythrocytes and hemosiderin deposits occasioning a resemblance to a lymphoproliferative disorder.
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Affiliation(s)
- Isabel Kolm
- Department of Dermatology, University Hospital Zurich, Switzerland
| | - Caroline Buset
- Department of Dermatology, University Hospital Zurich, Switzerland.,Dermatology Service, Cantonal Hospital Frauenfeld, Switzerland
| | - Ursula Flury
- Division of Dermatology, Lucerne Cantonal Hospital and Cancer Center, Lucerne, Switzerland
| | - Daniel Nosek
- Department of Medical Biosciences/Pathology, Umeå University, Umeå, Sweden
| | - Dmitry V Kazakov
- Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic
| | - Werner Kempf
- Department of Dermatology, University Hospital Zurich, Switzerland.,Kempf & Pfaltz Histologische Diagnostik, Zurich, Switzerland
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10
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Çaytemel C, Baykut B, Ağırgöl Ş, Caf N, Demir FT, Türkoğlu Z, Uzuner EG. Pigmented purpuric dermatosis: Ten years of experience in a tertiary hospital and awareness of mycosis fungoides in differential diagnosis. J Cutan Pathol 2021; 48:611-616. [PMID: 33368594 DOI: 10.1111/cup.13949] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2020] [Revised: 12/16/2020] [Accepted: 12/21/2020] [Indexed: 11/28/2022]
Abstract
BACKGROUND Pigmented purpuric dermatoses (PPD) can clinically mimic many diseases. Histopathology provides a definitive diagnosis. The aim of the study is to reveal the features of patients with PPD and to determine the disease frequency in the differential diagnosis, especially mycosis fungoides (MF). METHODS We retrospectively reviewed records of patients with PPD admitted to our hospital from January 2010 to May 2019. We studied the histopathological features of 127 patients, and performed pattern analysis on cases with a confirmed histopathologic diagnosis of PPD. Among the cases presenting with clinical features of PPD, but displaying different histopathological diagnoses, we focused on MF and tried to clarify the features of PPD-like MF. RESULTS Overall, 389 patients were admitted to our hospital with PPD symptoms. Of them, 262 patients were diagnosed clinically and a histopathological examination was performed in 127 patients. Of 127, 87 were diagnosed with PPD, and in the remaining 40, non-specific features (9.4%), vasculitis (6.2%), pityriasis rosea (4.7%), MF (3.9%), suspected-MF (1.5%), and other dermatoses (%5.5) were detected. The biopsy findings of two patients showed PPD, but during follow-up, the diagnosis of MF was established. CONCLUSIONS MF should be included in the differential diagnosis of PPD cases presenting with longstanding and widespread involvement.
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Affiliation(s)
- Ceyda Çaytemel
- Department of Dermatology and Venereology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
| | - Başak Baykut
- Department of Dermatology and Venereology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
| | - Şenay Ağırgöl
- Department of Dermatology and Venereology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
| | - Nazlı Caf
- Department of Dermatology and Venereology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
| | - Filiz Topaloğlu Demir
- Department of Dermatology and Venereology, Istanbul Medipol University, School of Medicine, Istanbul, Turkey
| | - Zafer Türkoğlu
- Department of Dermatology and Venereology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
| | - Esen Gül Uzuner
- Department of Pathology, Istanbul Haseki Training and Research Hospital, Istanbul, Turkey
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11
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Pukhalskaya T, Brown JA, Sills AA, Smoller BR. A Longstanding, Persistent and Recurrent Case of Cryptogenic Panniculitis. Case Rep Dermatol 2020; 12:199-208. [PMID: 33362504 PMCID: PMC7747046 DOI: 10.1159/000509605] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2020] [Accepted: 06/17/2020] [Indexed: 11/19/2022] Open
Abstract
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous T-cell lymphoma. There may be a significant histologic overlap with traumatic panniculitis and lupus profundus. We describe a 54-year-old woman who had received a diagnosis of SPTCL based upon a left parietal scalp biopsy 5 years earlier. This diagnosis was supported by immunohistochemistry (IHC) demonstrating a CD8+ predominant lymphocyte population in the subcutis. T-cell gene rearrangement studies were not performed at that time. The patient was treated and showed significant clinical improvement. When several tender erythematous subcutaneous nodules appeared on the upper back, left plantar surface and pretibial region, repeat biopsy was performed. Histology revealed a lobular and septal panniculitis with no vasculitis. The infiltrate contained abundant eosinophils and histiocytes not seen in the original biopsy specimen. IHC demonstrated a mixture of CD4+, CD8+ and CD7+ lymphocytes with abundant CD68+ histiocytes. T-cell gene rearrangement studies performed on one of the lesions failed to demonstrate clonality. It is important to recognize that patients with SPTCL are not exempt from other types of panniculitis, and complete histologic, IHC and molecular workups are essential to properly classify all cutaneous lesions in these patients.
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Affiliation(s)
- Tatsiana Pukhalskaya
- Department of Pathology and Laboratory Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, USA
| | - J Ahmad Brown
- Arkansas Dermatopathology, Little Rock, Arkansas, USA
| | | | - Bruce R Smoller
- Department of Pathology and Laboratory Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, USA
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12
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Gibson SE, Swerdlow SH. How I Diagnose Primary Cutaneous Marginal Zone Lymphoma. Am J Clin Pathol 2020; 154:428-449. [PMID: 32808967 DOI: 10.1093/ajcp/aqaa116] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
OBJECTIVES Primary cutaneous marginal zone lymphoma (PCMZL) is 1 of the 3 major subtypes of primary cutaneous B-cell lymphoma. The diagnosis of PCMZL may be challenging, as the differential diagnosis includes benign cutaneous lymphoproliferations as well as other primary or secondary cutaneous B-cell or T-cell lymphomas. This review describes our approach to the diagnosis of PCMZL. METHODS Two cases are presented that illustrate how we diagnose each of the 2 subtypes of PCMZL. The clinicopathologic features of PCMZL and the ways in which these cases can be distinguished from both benign and other neoplastic entities are emphasized. RESULTS A definitive diagnosis of PCMZL requires the incorporation of histologic and immunophenotypic features, molecular genetic studies in some cases, and just as importantly, clinical findings. Emerging data suggest that the heavy chain class-switched cases may be more like a clonal chronic lymphoproliferative disorder. CONCLUSIONS The 2 subtypes of PCMZL create different diagnostic challenges and require the use of a multiparameter approach. Although very indolent, it is important to distinguish PCMZLs from reactive proliferations, because they frequently recur and may require antineoplastic therapies. It is also critical to distinguish PCMZLs from other B- or T-cell lymphomas so that patients are properly evaluated and not overtreated.
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13
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Gökyayla E, Çetinarslan T, Temiz P, Türel Ermertcan A. Mycosis fungoides mimicking pigmented purpuric dermatosis. Dermatol Ther 2020; 33:e14062. [PMID: 32705758 DOI: 10.1111/dth.14062] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2020] [Revised: 07/17/2020] [Accepted: 07/21/2020] [Indexed: 11/30/2022]
Affiliation(s)
- Ece Gökyayla
- Medical Faculty, Department of Dermatology, Manisa Celal Bayar University, Manisa, Turkey
| | - Tubanur Çetinarslan
- Medical Faculty, Department of Dermatology, Manisa Celal Bayar University, Manisa, Turkey
| | - Peyker Temiz
- Medical Faculty, Department of Pathology, Manisa Celal Bayar University, Manisa, Turkey
| | - Aylin Türel Ermertcan
- Medical Faculty, Department of Dermatology, Manisa Celal Bayar University, Manisa, Turkey
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14
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Pires CAA, Pereira NG, Moreira AG, Sena JMC, Costa CCC, Bastos TDS, Rodrigues FDN, Faustino EDP, da Silva JRC, Dos Santos MAL. Cutaneous leishmaniasis mimicking cutaneous lymphoma. IDCases 2019; 17:e00580. [PMID: 31338298 PMCID: PMC6627646 DOI: 10.1016/j.idcr.2019.e00580] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2019] [Revised: 06/19/2019] [Accepted: 06/20/2019] [Indexed: 02/07/2023] Open
Abstract
Cutaneous leishmaniasis (CL) is caused by protozoa of Leishmania genus that are transmitted to humans through the bite of sand flies (Lutzomyia and Phlebotomus). The infection is classically manifested as multiple or single ulcers affecting cutaneous and/or mucosal areas of the body. Atypical lesions are relatively uncommon, being able to simulate a large variety of benign and malign dermatological disorders. In this article, we described a case of CL mimicking a clinical presentation of cutaneous lymphoma.
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Affiliation(s)
| | - Nathalia Gabay Pereira
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
| | - Amanda Gabay Moreira
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
| | - Julie Marie Costa Sena
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
| | - Caio César Chaves Costa
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
| | - Thalita Dos Santos Bastos
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
| | | | - Evelyn de Paiva Faustino
- Center for Biological and Health Sciences, Faculty of Medicine, Pará State University, Belém, Pará, Brazil
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15
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Demina OM, Akilov OE, Rumyantsev AG. Cutaneous T-cell lymphomas: modern data of pathogenesis, clinics and therapy. ONCOHEMATOLOGY 2018. [DOI: 10.17650/1818-8346-2018-13-3-25-38] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 04/11/2023]
Abstract
Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by skin infiltration with malignant monoclonal T lymphocytes. More common in adults aged 55 to 60 years, the annual incidence is about 0.5 per 100 000 people. Mycosis fungoides, Sézary syndrome and CD30+ lymphoproliferative diseases are the main subtypes of CTCL. To date, CTCL have a complex concept of etiopathogenesis, diagnosis, therapy and prognosis. The article presented summary data on these issues.
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Affiliation(s)
- O. M. Demina
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
| | - O. E. Akilov
- University of Pittsburgh, Department of Dermatology, Cutaneous Lymphoma Clinics
| | - A. G. Rumyantsev
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
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Pearson DR, Fujita M, High WA. Fulminant Mycosis Fungoides with Tissue Eosinophilia: A Unique Presentation of Two Cases with Acro-Periorbital Ulceration and An Aggressive Clinical Course. ACTA ACUST UNITED AC 2017; 8. [PMID: 28758048 PMCID: PMC5531183 DOI: 10.4172/2155-9929.1000337] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
We describe two unique cases of fulminant mycosis fungoides with remarkably similar and aggressive clinical courses resulting in death. Both cases demonstrated ulcerated palmar and periorbital plaques and marked tissue eosinophilia, which was confirmed by T-cell receptor γ chain gene rearrangement studies to display identical monoclonality at temporally and anatomically distinct sites. Dense eosinophilic infiltrates on biopsy led to misdiagnosis of inflammatory dermatoses in both instances. While mycosis fungoides may be challenging to diagnose histologically, the presence of eosinophils in progressive disease may herald a poor prognosis and should not exclude the diagnosis.
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Affiliation(s)
| | - Mayumi Fujita
- University of Colorado School of Medicine in Aurora, USA
| | - Whitney A High
- University of Colorado School of Medicine in Aurora, USA
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Benjamin Chase A, Markel K, Tawa MC. Optimizing Care and Compliance for the Treatment of Mycosis Fungoides Cutaneous T-Cell Lymphoma With Mechlorethamine Gel. Clin J Oncol Nurs 2017; 19:E131-9. [PMID: 26583648 DOI: 10.1188/15.cjon.e131-e139] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
BACKGROUND Mycosis fungoides is the most common form of cutaneous T-cell lymphoma (MF-CTCL). Quality nursing care is necessary for effective diagnosis and treatment of patients with MF-CTCL. Early-stage MF-CTCL (stages Ia and Ib) is most often managed in both dermatology and multidisciplinary settings. These stages can be effectively controlled by skin-directed therapies such as mechlorethamine gel (Valchlor®). Topical mechlorethamine has been used since the 1940s as an alkylating agent; however, compounded formulas have disadvantages that limit patient compliance. In contrast, newly approved mechlorethamine gel has demonstrated an efficacious and well-tolerated profile that has longer stability and is quicker to dry than its compounded counterpart. OBJECTIVES This article aims to provide recommendations for optimal nursing care of patients who have been diagnosed with stage Ia or Ib MF-CTCL. METHODS Four real-world patient cases are examined, along with practical considerations for the use of mechlorethamine gel to treat patients with MF-CTCL. FINDINGS Nurses can promote patient adherence through specific interventions and strategies, such as education about mechlorethamine gel, its mechanism of action, and safety and efficacy, as well as connecting patients with patient assistance programs or other supportive services.
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Kelati A, Gallouj S, Tahiri L, Harmouche T, Mernissi FZ. Defining the mimics and clinico-histological diagnosis criteria for mycosis fungoides to minimize misdiagnosis. Int J Womens Dermatol 2017; 3:100-106. [PMID: 28560304 PMCID: PMC5440453 DOI: 10.1016/j.ijwd.2016.11.006] [Citation(s) in RCA: 26] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2016] [Revised: 11/18/2016] [Accepted: 11/21/2016] [Indexed: 01/13/2023] Open
Abstract
Background Mycosis fungoides (MF) is a significant diagnostic challenge; it has various differential diagnosis especially at an early stage. Our aim was to describe mimics of MF clinically and histologically, and to define significant diagnostic criteria of the disease. Methods This was a retro-prospective cohort of 370 patients in whom the diagnosis of MF was suspected clinically. Results MF was histologically confirmed in 15.4% of cases and rejected in 84.5%. Other identified histologically diagnosis were eczema, psoriasis; nonspecific dermatitis, lichen, lupus; pseudolymphoma, parapsoriasis and toxidermia. 4 patients with palmoplantar MF were wrongly treated as eczema, and 10 patients with psoriasiform MF were initially treated as psoriasis. We also described the clinical, histological and immunohistochemistry diagnostic criteria for distinguishing MF from benign dermatosis. Conclusions Misdiagnosis of MF was a real problem for this study, because it shared common clinical and histological characteristics with other inflammatory diseases like eczema and psoriasis. Therefore, defining significant clinico-histological diagnosis criteria of MF would be of great help and would increase the accuracy of the diagnosis.
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Affiliation(s)
- A Kelati
- Department of Dermatology, Faculty of Medicine, Hospital Hassan II, Fez, Morocco
| | - S Gallouj
- Department of Dermatology, Faculty of Medicine, Hospital Hassan II, Fez, Morocco
| | - L Tahiri
- Department of Anatomopathology, Faculty of Medicine, Hospital Hassan II, Fez, Morocco
| | - T Harmouche
- Department of Anatomopathology, Faculty of Medicine, Hospital Hassan II, Fez, Morocco
| | - F Z Mernissi
- Department of Dermatology, Faculty of Medicine, Hospital Hassan II, Fez, Morocco
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Abstract
Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Microscopic findings from 2 biopsies included lymphocytes at the dermoepidermal junction with mild interface dermatitis, a dense lymphocytic infiltrate that was concentrated around adnexae and subcutaneous fat in concert with thickened collagen bundles and mild widening of fibrous septae surrounding fat lobules. Although the clinical differential diagnosis included panniculitis or a sporotrichoid infection, 1 biopsy showed a dense lymphocytic infiltrate histologically bordered on that of cutaneous lymphoid hyperplasia or a late stage of Lyme disease, and a second also demonstrated more prominent sclerodermoid collagen bundles rendering the diagnosis of linear sclerodermoid LE profundus.
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Abstract
Cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ T-LPD) represent a spectrum encompassing lymphomatoid papulosis (LyP), primary cutaneous anaplastic large-cell lymphoma (pcALCL) and borderline lesions. They share the expression of CD30 as a common phenotypic marker. They differ however in their clinical presentation, the histological features and clinical course. Moreover, LyP and PcALCL show numerous clinical, histological and phenotypic variants. Overlapping features of LyP and pcALCL with themselves and with other cutaneous and systemic lymphomas emphasize the importance of careful clinicopathologic correlation and staging in the diagnosis of CD30+ T-LPD. Furthermore, an increasing number of inflammatory and infectious skin disorders harboring medium-sized to large CD30+ cells have to be considered in the differential diagnosis. Whereas the expression of CD30 in cutaneous CD30+ T-LPD stands for a favourable prognosis, its expression in other cutaneous and systemic lymphomas has a divergent impact. The assessment of CD30 expression does not only provide prognostic information, but is of potential therapeutic relevance as CD30 can serve as a therapeutic target. This review focuses on the clinicopathological and phenotypic spectrum of CD30+ T-LPD, its differential diagnoses and the role of CD30 as a diagnostic, prognostic and therapeutic marker.
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Affiliation(s)
- Werner Kempf
- Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland; Department of Dermatology, University Hospital Zurich, CH-8091, Zurich, Switzerland.
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Abstract
Primary cutaneous marginal zone lymphoma (CMZL) is one of the major primary B-cell lymphomas of skin. Two types are recognized: a more common class-chain switched CMZL, and a much less common IgM+ CMZL. The extremely indolent course, together with other features distinct from most other MALT lymphomas, has led some to question whether at least the class-switched cases should be considered an overt lymphoma.
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Affiliation(s)
- Steven H Swerdlow
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, UPMC-Presbyterian, G-335, 200 Lothrop Street, Pittsburgh, PA 15213.
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22
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Charli-Joseph YV, Gatica-Torres M, Pincus LB. Approach to Cutaneous Lymphoid Infiltrates: When to Consider Lymphoma? Indian J Dermatol 2016; 61:351-74. [PMID: 27512181 PMCID: PMC4966394 DOI: 10.4103/0019-5154.185698] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Cutaneous lymphoid infiltrates (CLIs) are common in routine dermatopathology. However, differentiating a reactive CLI from a malignant lymphocytic infiltrate is often a significant challenge since many inflammatory dermatoses can clinically and/or histopathologically mimic cutaneous lymphomas, coined pseudolymphomas. We conducted a literature review from 1966 to July 1, 2015, at PubMed.gov using the search terms: Cutaneous lymphoma, cutaneous pseudolymphoma, cutaneous lymphoid hyperplasia, simulants/mimics/imitators of cutaneous lymphomas, and cutaneous lymphoid infiltrates. The diagnostic approach to CLIs and the most common differential imitators of lymphoma is discussed herein based on six predominant morphologic and immunophenotypic, histopathologic patterns: (1) Superficial dermal T-cell infiltrates (2) superficial and deep dermal perivascular and/or nodular natural killer/T-cell infiltrates (3) pan-dermal diffuse T-cell infiltrates (4) panniculitic T-cell infiltrates (5) small cell predominant B-cell infiltrates, and (6) large-cell predominant B-cell infiltrates. Since no single histopathological feature is sufficient to discern between a benign and a malignant CLI, the overall balance of clinical, histopathological, immunophenotypic, and molecular features should be considered carefully to establish a diagnosis. Despite advances in ancillary studies such as immunohistochemistry and molecular clonality, these studies often display specificity and sensitivity limitations. Therefore, proper clinicopathological correlation still remains the gold standard for the precise diagnosis of CLIs.
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Affiliation(s)
- Yann Vincent Charli-Joseph
- Cutaneous Hematopathology Clinic, Department of Dermatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Michelle Gatica-Torres
- Cutaneous Hematopathology Clinic, Department of Dermatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
| | - Laura Beth Pincus
- Department of Dermatology and Pathology, University of California, San Francisco, United States of America
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Riyaz N, Sasidharanpillai S, Abdul Latheef EN, Davul H, Ashraf F. Pigmented purpuric dermatosis or mycosis fungoides: A diagnostic dilemma. Indian Dermatol Online J 2016; 7:183-5. [PMID: 27294054 PMCID: PMC4886591 DOI: 10.4103/2229-5178.182361] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Pigmented purpuric dermatoses (PPD), a group of vascular disorders with variable clinical picture is reported in all races and age groups with a male predilection. There are reports of mycosis fungoides manifesting as pigmented purpura as well as progression of PPD to cutaneous T-cell lymphoma. The diagnostic dilemma is compounded by PPD manifesting histological similarity to mycosis fungoides. Currently, it is believed that PPD with monoclonal T-cell population is more likely to progress to malignancy. We report a 31-year-old male patient who presented with the lichenoid clinical variant of PPD lesions that mimicked mycosis fungoides on histopathology. Gene rearrangement studies identified a polyclonal T-cell population. The patient responded to photochemotherapy, which is beneficial in both PPD and mycosis fungoides. Our case signifies the limitations of current diagnostic modalities in accurately distinguishing PPD from cutaneous lymphoma. Data on disease progression in similar cases may enable us to formulate better diagnostic definitions.
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Affiliation(s)
- Najeeba Riyaz
- Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
| | - Sarita Sasidharanpillai
- Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
| | | | - Hena Davul
- Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
| | - Febin Ashraf
- Department of Dermatology and Venereology, Government Medical College, Kozhikode, Kerala, India
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Mathew LM, Kapila R, Schwartz RA. Kikuchi-Fujimoto disease: a diagnostic dilemma. Int J Dermatol 2016; 55:1069-75. [PMID: 27207311 DOI: 10.1111/ijd.13314] [Citation(s) in RCA: 29] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2015] [Revised: 12/08/2015] [Accepted: 02/03/2016] [Indexed: 11/30/2022]
Abstract
Kikuchi-Fujimoto disease is a benign, self-limiting condition characterized by lymphadenopathy and fever. Its diagnosis can be challenging, as the disease must be differentiated from lymphoma and systemic lupus erythematosus. Patients can present with nonspecific cutaneous lesions. Adequate clinical and histopathologic correlation can aid in the timely diagnosis of Kikuchi's disease, thus preventing the use of unnecessary diagnostic procedures and inappropriate treatments.
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Affiliation(s)
- Laju M Mathew
- Dermatology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA
| | - Rajendra Kapila
- Infectious Diseases, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.,Medicine, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA
| | - Robert A Schwartz
- Dermatology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Medicine, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Pathology, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Pediatrics, Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA. .,Rutgers New Jersey Medical School and Rutgers University School of Public Affairs and Administration, Newark, NJ, USA.
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25
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Vonderheid EC, Kadin ME, Telang GH. Commentary about papular mycosis fungoides, lymphomatoid papulosis and lymphomatoid pityriasis lichenoides: more similarities than differences. J Cutan Pathol 2015; 43:303-12. [PMID: 26566599 DOI: 10.1111/cup.12653] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2015] [Revised: 11/04/2015] [Accepted: 10/07/2015] [Indexed: 12/24/2022]
Affiliation(s)
- Eric C Vonderheid
- Sidney Kimmel Cancer Center, Johns Hopkins Medical Institutes, Baltimore, MD, USA
| | - Marshall E Kadin
- Department of Dermatology, Boston University and Roger Williams Medical Center, Providence, RI, USA
| | - Gladys H Telang
- Department of Dermatology, The Warren Alpert Medical School of Brown University, Providence, RI, USA
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Riyaz N, Sasidharanpillai S, Aravindan KP, Nobin BK, Raghavan NT, Nikhila PK. Phenytoin Induced Cutaneous B Cell Pseudolymphoma. Indian J Dermatol 2015; 60:522. [PMID: 26538730 PMCID: PMC4601451 DOI: 10.4103/0019-5154.164437] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Abstract
Cutaneous pseudolymphomas are benign lymphoproliferative processes mimicking lymphomas clinically and histologically. One of the precipitating factors for pseudolymphoma is drugs like anticonvulsants, antidepressants and angiotensin-converting enzyme inhibitors. According to existing literature phenytoin-induced cutaneous pseudolymphomas are usually T-cell predominant. Most often withdrawal of the drug with or without short-course systemic steroids can attain a cure. Rarely malignant transformation has been reported years later despite withdrawal of the offending drug, which necessitates a long-term follow up of the affected. We report an 80-year-old male patient who was receiving phenytoin sodium and who presented with diffuse erythema and infiltrated skin lesions which histologically resembled cutaneous B-cell lymphoma. Substituting phenytoin with levetiracetam achieved resolution of symptoms. Further evaluation was suggestive of a reactive process. A detailed drug history is of paramount importance in differentiating drug-induced pseudolymphoma from lymphoma. Searching literature we could not find any previous reports of phenytoin-induced cutaneous B-cell pseudolymphoma.
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Affiliation(s)
- Najeeba Riyaz
- Department of Dermatology and Venereology, Govt Medical College, Kozhikode, Kerala, India
| | | | | | - Babu K Nobin
- Department of Pathology, Govt Medical College, Kozhikode, Kerala, India
| | - Nisha T Raghavan
- Department of Pathology, Govt Medical College, Kozhikode, Kerala, India
| | - Pappinissery K Nikhila
- Department of Dermatology and Venereology, Govt Medical College, Kozhikode, Kerala, India
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27
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Wobser M, Geissinger E, Rosenwald A, Goebeler M. Mycosis fungoides: A mimicker of benign dermatoses. World J Dermatol 2015; 4:135-144. [DOI: 10.5314/wjd.v4.i4.135] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/22/2014] [Revised: 08/21/2014] [Accepted: 07/17/2015] [Indexed: 02/06/2023] Open
Abstract
Mycosis fungoides, the most common primary cutaneous lymphoma, may present with a broad spectrum of clinical features. As both clinical and dermatopathological findings in mycosis fungoides occasionally closely imitate other dermatoses, correct diagnosis may be a challenge both for clinicians as well as dermatopathologists. As a consequence, diagnosis of cutaneous lymphoma may be initially missed and, therefore, prompt and adequate therapeutic measures delayed. Hence, the purpose of our article was to give an overview of hitherto published “mimickers” of mycosis fungoides with a review of its diverse clinical features to alert the clinicians about the wide spectrum of this dissimulating disease. By integrating our own encountered atypical cases of mycosis fungoides we provide a comprehensive illustrated histological and moleculargenetic workup thereof and thereby critically revise the different available diagnostic tools of daily routine. Finally, we derive a practical algorithm to obtain the correct diagnosis even in such ambiguous cases of mycosis fungoides.
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28
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Deaver D, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control 2015; 21:313-21. [PMID: 25310212 DOI: 10.1177/107327481402100407] [Citation(s) in RCA: 69] [Impact Index Per Article: 6.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is associated with the development of systemic lupus erythematosus (SLE). METHODS The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms. RESULTS Although KFD has been reported in other ethnic groups and geographical areas, it is more frequently diagnosed in young women of Asian descent. Patients with the disease typically present with rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, and headache. Diagnosis is based on histopathological examination. Excisional lymph node biopsy is essential for a correct diagnosis. Apoptotic coagulation necrosis with karyorrhectic debris and the proliferation of histiocytes, plasmacytoid dendritic cells, and CD8(+) T cells in the absence of neutrophils are characteristic cytomorphology features. Interface dermatitis at the onset of KFD may be a marker for the subsequent evolution of SLE. The natural course of the disease is typically benign. Short courses of steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can be administered to patients with more severe symptoms. CONCLUSIONS Although KFD was described more than 40 years ago, the etiology of this disease remains unsolved. Infectious or autoimmune processes were proposed but have not been definitively confirmed. Clinical presentation with systemic B symptoms and adenopathy may lead to an erroneous diagnosis of malignant lymphoma. The introduction of modern methods into hematopathology, including immunohistochemistry, flow cytometry, and molecular clonality studies, has decreased the probability of misdiagnosis. Until reliable prognostic markers are available, patients with KFD should have continued long-term follow-up care due to their increased risk of SLE.
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Dewar R, Andea AA, Guitart J, Arber DA, Weiss LM. Best practices in diagnostic immunohistochemistry: workup of cutaneous lymphoid lesions in the diagnosis of primary cutaneous lymphoma. Arch Pathol Lab Med 2015; 139:338-50. [PMID: 25724031 DOI: 10.5858/arpa.2014-0018-cp] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
CONTEXT Primary cutaneous lymphoma is a common extranodal non-Hodgkin lymphoma. These lesions share common features with their nodal counterparts, but also have differences in morphology, unique clinical presentations, and immunohistochemical features. OBJECTIVE To review the 2008 World Health Organization (WHO) and 2005 consensus WHO-EORTC (European Organisation for Research and Treatment of Cancer) classifications, and address the immunohistochemical findings in the most common primary cutaneous T- and B-cell lymphomas. Since clonality testing is commonly used as an ancillary test in the evaluation of cutaneous lymphoma, a brief section in the use and pitfalls of clonality testing is included. DATA SOURCES The WHO and EORTC classification publications and the relevant recent literature were used to compile appropriate and practical guidelines in this review. CONCLUSIONS The practice of dermatopathology and hematopathology varies widely. Thus, while this review provides an overview and guideline for the workup of lymphoid lesions of the skin, the practitioner should understand the importance of clinical correlation as well as appropriate utility of available resources (such as clonality testing) in arriving at a diagnosis in cutaneous lymphoid lesions.
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Affiliation(s)
- Rajan Dewar
- From the Department of Pathology, Beth Israel Deaconess Medical Center, and Harvard Medical School, Boston, Massachusetts (Dr Dewar); the Department of Pathology, University of Michigan, Ann Arbor (Dr Andea); the Department of Pathology and Dermatology, Northwestern University, Chicago, Illinois (Dr Guitart); the Department of Pathology, Stanford University Medical Center, Stanford, California (Dr Arber); and the Department of Hematopathology, Clarient Pathology Services, Inc, Aliso Viejo, California (Dr Weiss)
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Lobular Panniculitic Infiltrates With Overlapping Histopathologic Features of Lupus Panniculitis (Lupus Profundus) and Subcutaneous T-cell Lymphoma. Am J Surg Pathol 2015; 39:206-11. [DOI: 10.1097/pas.0000000000000307] [Citation(s) in RCA: 50] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Arps DP, Chen S, Fullen DR, Hristov AC. Selected Inflammatory Imitators of Mycosis Fungoides: Histologic Features and Utility of Ancillary Studies. Arch Pathol Lab Med 2014; 138:1319-27. [DOI: 10.5858/arpa.2014-0298-cc] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Mycosis fungoides is the most common primary cutaneous lymphoma; however, it remains a significant diagnostic challenge, in part because of the overlap with several inflammatory dermatoses. Despite advances in immunohistochemistry and molecular diagnostics, false-positive, false-negative, and indeterminate diagnoses are not uncommon. In most cases, the overall balance of morphologic, immunophenotypic, and genetic features must be considered carefully because there are few sensitive and specific clues to the diagnosis. Moreover, an appropriate clinical presentation is essential to the diagnosis and helps to favor or exclude inflammatory/reactive processes. Herein, we discuss 3 important inflammatory dermatoses that may closely simulate mycosis fungoides, and we review the use of ancillary studies in these challenging cases.
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Affiliation(s)
- David P. Arps
- From the Departments of Pathology (Drs Arps, Chen, Fullen, and Hristov) and Dermatology (Drs Fullen and Hristov), University of Michigan, Ann Arbor
| | - Stephanie Chen
- From the Departments of Pathology (Drs Arps, Chen, Fullen, and Hristov) and Dermatology (Drs Fullen and Hristov), University of Michigan, Ann Arbor
| | - Douglas R. Fullen
- From the Departments of Pathology (Drs Arps, Chen, Fullen, and Hristov) and Dermatology (Drs Fullen and Hristov), University of Michigan, Ann Arbor
| | - Alexandra C. Hristov
- From the Departments of Pathology (Drs Arps, Chen, Fullen, and Hristov) and Dermatology (Drs Fullen and Hristov), University of Michigan, Ann Arbor
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Abstract
Cutaneous CD30+ lymphoproliferative disorders are the second most common types of cutaneous T-cell lymphomas. They represent a well-defined spectrum encompassing lymphomatoid papulosis (LyP), primary cutaneous anaplastic large-cell lymphoma (pcALCL), and borderline lesions. They share the expression of CD30 as a common phenotypic hallmark, but they differ in their clinical presentation, course, and histologic features. New variants have been recently identified, including CD8+ epidermotropic LyP type D, angioinvasive LyP type E, and ALK-positive pcALCL. This review describes clinical, histopathologic, and phenotypic variants; their differential diagnoses (benign and malignant); and the role of CD30 as a diagnostic, prognostic, and therapeutic marker.
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Affiliation(s)
- Werner Kempf
- Kempf und Pfaltz, Histologische Diagnostik, Seminarstrasse 1, Zürich CH-8042, Switzerland; Department of Dermatology, University Hospital, Zürich CH-8091, Switzerland.
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Abstract
OBJECTIVES The objectives of this study are to review prior publications of survival for patients with mycosis fungoides (MF), to perform some analyses on the consolidated data, and then to consider the implications of the results. METHODS The data for this study comprise 18 survival curves derived from seven prior publications of long-term survival in relatively large series of patients with MF. Altogether, the study uses results from over 5,000 patients. To examine fatality, the study uses hazard functions derived from the survival curves. RESULTS The analyses demonstrate significant variability in survival between different groups who have studied MF, and the results document that for most patients the diagnosis of MF has little impact on fatality. CONCLUSIONS Although MF is considered a cutaneous lymphoma, that is, a malignancy, it may be time to reconsider low stages of MF as precursors to malignancy analogous to the precursors to malignancies of other types or organs.
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Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome). J Am Acad Dermatol 2014; 70:205.e1-16; quiz 221-2. [DOI: 10.1016/j.jaad.2013.07.049] [Citation(s) in RCA: 171] [Impact Index Per Article: 15.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2013] [Revised: 06/25/2013] [Accepted: 07/01/2013] [Indexed: 02/08/2023]
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Attygalle AD, Cabeçadas J, Gaulard P, Jaffe ES, de Jong D, Ko YH, Said J, Klapper W. Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology. Histopathology 2013; 64:171-99. [PMID: 24128129 DOI: 10.1111/his.12251] [Citation(s) in RCA: 121] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Abstract
Mature T-cell and T/NK-cell neoplasms are both uncommon and heterogeneous, among the broad category of non-Hodgkin lymphomas. Owing to the lack of specific genetic alterations in the vast majority, most currently defined entities show overlapping morphological and immunophenotypic features, and therefore pose a challenge to the diagnostic pathologist. In the light of recent immunophenotypic, cytogenetic and molecular genetics advances in the field of T-cell and T/NK-cell lymphomas, the focus of the lymphoma workshop of the European Association for Haematopathology/Society for Hematopathology meeting in Lisbon, Portugal, in October 2012 was to refine existing diagnostic criteria and clarify the borders between overlapping entities. The panel reviewed over 200 submitted cases, which were grouped into five categories: (i) angioimmunoblastic T-cell lymphoma and T-follicular-helper-cell-associated lymphomas; (ii) CD30-positive T-cell lymphomas/lymphoproliferative diseases; (iii) extranodal T-cell and NK-cell neoplasms; (iv) EBV-associated T-cell/NK-cell lymphomas/lymphoproliferative diseases; and (v) peripheral T-cell lymphoma, not otherwise specified, post-transplant lymphoproliferative disorders, and mimics. This report summarizes the discussions and conclusions of the workshop, which question current diagnostic criteria and provide recommendations for refining existing classifications.
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Quintanilla-Martinez L, Jansen PM, Kinney MC, Swerdlow SH, Willemze R. Non-mycosis fungoides cutaneous T-cell lymphomas: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol 2013; 139:491-514. [PMID: 23525618 DOI: 10.1309/ajcp83aoqtmlojtm] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
Abstract
Primary cutaneous T-cell lymphomas (CTCL) excluding mycosis fungoides (MF) were discussed in 2 sessions of the 2011 Society for Hematopathology/ European Association of Haematopathology Workshop, Los Angeles, CA. Session 2 focused on primary cutaneous CD30+ T-cell lymphoproliferative disorders and their differential diagnosis, including systemic CD30+ T-cell lymphoma secondarily infiltrating the skin. Interesting features like special morphologic variants and atypical phenotypes were presented. In addition, the possibility of rare ALK+ primary cutaneous lymphomas was discussed. Session 3 examined other more uncommon non-MF CTCLs, including subcutaneous panniculitis-like T-cell lymphoma, extranodal NK/T-cell lymphoma, hydroa vacciniforme-like T-cell lymphoma, and rare subtypes of primary cutaneous peripheral T-cell lymphoma, not otherwise specified. In addition, systemic T-cell lymphomas involving the skin secondarily, such as angioimmunoblastic T-cell lymphoma, were included in this session. In this report, novel findings, areas of special interest, and diagnostic challenges emerging from the cases submitted to the workshop will be highlighted. The necessity to integrate histologic, immunophenotypical, genetic, and in particular, clinical data to arrive at the correct diagnosis, and subsequently provide adequate treatment, is emphasized.
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Affiliation(s)
- Leticia Quintanilla-Martinez
- Institute of Pathology, and Comprehensive Cancer Center (CCC), Eberhard-Karls-University of Tübingen, Tübingen, Germany
| | - Patty M. Jansen
- Departments of Pathology, Leiden University Medical Center, Leiden, The Netherlands
| | - Marsha C. Kinney
- Department of Pathology, University of Texas Health Science Center at San Antonio
| | - Steven H. Swerdlow
- Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA
| | - Rein Willemze
- Dermatology, Leiden University Medical Center, Leiden, The Netherlands
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Song SX, Willemze R, Swerdlow SH, Kinney MC, Said JW. Mycosis fungoides: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol 2013; 139:466-90. [PMID: 23525617 DOI: 10.1309/ajcpobdp2oqaj5br] [Citation(s) in RCA: 50] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022] Open
Abstract
Session 1 of the 2011 Workshop of the Society for Hematopathology and European Association for Haematopathology focused on mycosis fungoides (MF), the most common cutaneous lymphoma. The 62 cases in this case group demonstrated a wide spectrum of clinicopathologic features, including those seen in typical cases as well as those, by contrast, with atypical clinical history, morphology, immunophenotype, and/or genotype. Of the 62 cases, 27 (44%) were presented at the workshop and highlighted diagnostic challenges plus related issues. This report summarizes the approach recommended for making a confident diagnosis of MF and its clinically significant variants; emphasizes pitfalls in evaluating early MF, assessing nodal involvement, and diagnosing transformed MF; and discusses the relationship between MF and primary cutaneous CD30+ T-cell lymphoproliferative disorders. Last, Sézary syndrome is discussed, with concentration on those features distinct from MF.
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Affiliation(s)
- Sophie X. Song
- Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA
| | - Rein Willemze
- Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands
| | - Steven H. Swerdlow
- Department of Pathology, Division of Hematopathology, University of Pittsburgh School of Medicine, Pittsburgh, PA
| | - Marsha C. Kinney
- Department of Pathology, University of Texas Health Science Center, San Antonio, TX
| | - Jonathan W. Said
- Department of Pathology and Laboratory Medicine, UCLA Medical Center, Los Angeles, CA
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Swerdlow SH, Quintanilla-Martinez L, Willemze R, Kinney MC. Cutaneous B-cell lymphoproliferative disorders: report of the 2011 Society for Hematopathology/European Association for Haematopathology workshop. Am J Clin Pathol 2013; 139:515-35. [PMID: 23525619 DOI: 10.1309/ajcpnlc9nc9wtqyy] [Citation(s) in RCA: 32] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/04/2023] Open
Abstract
The diagnosis and classification of the cutaneous B-cell lymphomas can be quite a challenge, with a definitive diagnosis sometimes being elusive, even when an extensive workup has been performed. Distinction of benign from neoplastic disorders can be difficult, with some hyperplasias mimicking lymphomas and vice versa. There are only a limited number of skin-specific B-cell lymphomas, including primary cutaneous follicle center lymphoma and primary cutaneous diffuse large B-cell lymphoma, leg type. Cutaneous marginal zone lymphomas have distinctive features but are classified with the other mucosa-associated lymphoid tissue lymphomas. It is important, however, to also remember that many other B-cell lymphomas/ plasma cell neoplasms can primarily, or more often secondarily, involve the skin. Some may mimic one of the skin-specific lymphomas but have very different clinical implications. Iatrogenic and senescent immunodeficiency-associated lymphoproliferative disorders that are often Epstein-Barr virus (EBV) positive can also primarily involve the skin, including cases also known as EBV-positive mucocutaneous ulcer.
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Affiliation(s)
- Steven H. Swerdlow
- Department of Pathology, Division of Hematopathology, University of Pittsburgh School of Medicine, Pittsburgh, PA
| | | | - Rein Willemze
- Department of Dermatology, Leiden University Medical Center, Leiden, the Netherlands
| | - Marsha C. Kinney
- Department of Pathology, University of Texas Health Science Center, San Antonio, TX
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