• Autoantibody
• Autoimmune bullous diseases
• Immunogenic domain
• Inflammation
• Pathogenesis
• Targeted therapy

• Humans
• Autoimmune Diseases/therapy
• Autoimmune Diseases/diagnosis
• Autoimmune Diseases/etiology
• Autoimmune Diseases/immunology
• Autoimmune Diseases/pathology
• Skin Diseases, Vesiculobullous/therapy
• Skin Diseases, Vesiculobullous/diagnosis
• Skin Diseases, Vesiculobullous/etiology
• Skin Diseases, Vesiculobullous/immunology
• Skin Diseases, Vesiculobullous/pathology
• Autoantibodies/immunology
• Disease Management
• Animals

• 82304062 the National Natural Science Foundation of China
• 2024NSFSC1626 the Natural Science Foundation of Sichuan Province
• 2023SCU12065 the Postdoctoral Fund of Sichuan University
• 2024HXBH182 Innovative Research Group Project of the National Natural Science Foundation of China
• 2024M752267 Sichuan Provincial Postdoctoral Science Foundation
• 2023-YF09-00003-SN the Achievement Transformation Demonstration Project of the Key research and development (R&D) Program of Science & Technology Department of Chengdu
• 2023HXBH098 the Postdoctoral Research Fund of West China Hospital, Sichuan University
• 2023NSFSC1551 Natural Science Foundation of Sichuan Province

• ELISA
• PAMS
• criteria
• diagnosis
• histopathology
• paraneoplastic
• paraneoplastic autoimmune syndrome
• pemphigus
• therapy
• treatment

• Humans
• Pemphigus/diagnosis
• Pemphigus/therapy
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/etiology
• Autoimmune Diseases/diagnosis
• Autoimmune Diseases/complications
• Autoimmune Diseases/therapy
• Multiple Organ Failure/etiology
• Multiple Organ Failure/diagnosis
• Immunosuppressive Agents/therapeutic use
• Male

• FOR 2497 Deutsche Forschungsgemeinschaft

• Humans
• Pemphigus/diagnosis
• Pemphigus/drug therapy
• Pemphigoid, Bullous/diagnosis
• Pemphigoid, Bullous/drug therapy

• FOR 2497 Deutsche Forschungsgemeinschaft

• paraneoplastic pemphigus
• paraneoplastic autoimmune multiorgan syndrome
• haematological malignancies
• castleman disease
• rituximab

• Animals
• Rats
• Autoimmune Diseases
• Neoplasms/complications
• Paraneoplastic Syndromes/diagnosis
• Paraneoplastic Syndromes/etiology
• Paraneoplastic Syndromes/therapy
• Paraneoplastic Syndromes, Nervous System/diagnosis
• Paraneoplastic Syndromes, Nervous System/etiology
• Paraneoplastic Syndromes, Nervous System/therapy
• Societies, Medical

• P30 CA093373 NCI NIH HHS

• IVIG
• Pemphigus Disease Activity Index
• azathioprine
• dapsone
• intraepithelial autoimmune bullous dermatoses
• mycophenolate mofetil
• paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome
• pemphigus erythematosus
• pemphigus foliaceus
• pemphigus herpetiformis
• pemphigus vulgaris
• rituximab

Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. PNP presents most frequently between 45 and 70 years old, but it also occurs in children and adolescents. A wide variety of lesions (florid oral mucosal lesions, a generalized polymorphous cutaneous eruption, and pulmonary involvement) may occur in patients with PNP. The earliest and most consistent finding is severe stomatitis. There is a spectrum of at least five clinical variants with different morphology. Similarly, the histological findings are very variable. Investigations to diagnose PNP should include checking for systemic complications (to identify tumor), skin biopsies (for histopathological and immunofluorescence studies), and serum immunological studies. PNP is characterized by the presence of autoantibodies against antigens such as desmoplakin I (250 kD), bullous pemphigoid aniygen I (230 kD), desmoplakin II (210 kD), envoplakin (210 kD), periplakin (190 kD), plectin (500 kD), and a 170 kD protein. Unlike other forms of pemphigus, PNP can affect other types of epithelia, such as gastrointestinal and respiratory tract. Treatment of PNP is difficult, and the best outcomes have been reported with benign neoplasms that have been surgically excised. The first-line treatment is high-dose corticosteroids with the addition of steroid-sparing agents. Treatment failures are often managed with rituximab with or without concomitant intravenous immunoglobulin. In general, the prognosis is poor, not only because of eventual progression of malignant tumors but also because treatment with aggressive immunosuppression therapy often results in infectious complications, which is unfortunately at this time the most common cause of death in PNP.

• direct and indirect immunofluorescence
• mucositis
• paraneoplastic pemphigus
• rituximab

The ocular presentation of Castleman’s disease (CD)-associated paraneoplastic pemphigus (PNP) has rarely been reported. In this report, we describe a young patient with CD-associated PNP who had recurrent corneal ulceration in addition to cicatrizing conjunctivitis.

We describe a case of 23-year-old male with mucocutaneous erosion and conjunctival injection and erosion who was found to have PNP. Pelvic hyaline-vascular CD was detected and completely excised. The mucocutaneous lesions improved postoperatively. Two years after pelvic surgery, the patient gradually developed conjunctival symblepharon in both eyes and pterygium in the right eye. The patient then underwent a successful exclusion of the symblepharon, an excision of the pterygium and an amniotic membrane transplantation in the right eye. However, after 6 months, he experienced an aseptic corneal ulcer and recurrent pterygiumin the right eye. After treatment with systemic and local immunosuppressive medications, the corneal ulcer gradually healed and remained stable.

Corneal ulceration and melting, in addition to conjunctivitis, as a complication of CD-associated PNP, can be successfully managed with systemic and local immunosuppressants.

• Castleman’s disease
• Corneal melting
• Paraneoplastic pemphigus

Pseudopemphigoid can cause a chronic cicatricial conjunctivitis that is clinically identical to the manifestations seen in mucous membrane pemphigoid, a disorder with a common clinical phenotype and multiple autoimmune links. For the purpose of this review, we will describe pseudopemphigoid as caused by topical drugs, the most common etiology with ocular manifestations, and as caused by the pemphigus disease, a more rare etiology. Specifically, we will discuss the ophthalmological features of drug-induced cicatricial conjunctivitis, pemphigus vulgaris, and paraneoplastic pemphigus. Other etiologies of pseudopemphigoid exist that will not be described in this review including autoimmune or inflammatory conditions such as lichen planus, sarcoidosis, granulomatosis with polyangiitis (Wegener’s granulomatosis), erythema multiforme (minor, major, and Stevens-Johnson syndrome), bullous pemphigoid, skin-dominated linear IgA bullous dermatosis, and skin-dominated epidermolysis bullosa acquisita. Prompt diagnosis of the underlying etiology in pseudopemphigoid is paramount to the patient’s outcome as certain diseases are associated with a more severe clinical course, increased ocular involvement, and differential response to treatment. A complete history and ocular examination may find early cicatricial changes in the conjunctiva that are important to note and evaluate to avoid progression to more severe disease manifestations. When such cicatricial changes are noted, proper diagnostic techniques are needed to help elucidate a diagnosis. Lastly, collaboration between ophthalmologists and subspecialists such as dermatologists, pathologists, immunologists, and others involved in the care of the patient is needed to ensure optimal management of disease.

• Pseudopemphigoid
• Mucous membrane pemphigoid
• Cicatricial conjunctivitis
• Pemphigus vulgaris
• Paraneoplastic pemphigus
• Drug-induced conjunctival cicatrization

The pemphigus group comprises the autoimmune intraepidermal blistering diseases classically divided into two major types: pemphigus vulgaris and pemphigus foliaceous. Pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus are rarer forms that present some clinical, histological and immunopathological characteristics that are different from the classical types. These are reviewed in this article. Future research may help definitively to locate the position of these forms in the pemphigus group, especially with regard to pemphigus herpetiformis and the IgG/ IgA pemphigus.