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Severino-Freire M, Granier Tournier C, Chiaverini C, Audouze A, Morice-Picard F, Texier H, Dreyfus I, Bing-Lecointe AC, Mallet S, Bodemer C, Fischer J, Jonca N, Mazereeuw-Hautier J. French national protocol for the management of congenital ichthyosis. Ann Dermatol Venereol 2024; 151:103247. [PMID: 38513308 DOI: 10.1016/j.annder.2024.103247] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2023] [Accepted: 09/27/2023] [Indexed: 03/23/2024]
Abstract
Congenital ichthyoses (CI) comprise a heterogeneous group of monogenic genetic skin diseases characterized by diffuse scaling, often associated with skin inflammation. Diagnosis of the individual form of ichthyosis is complex and is guided by clinical expertise. CI usually has a major impact on quality of life (QOL) and thus requires lifelong treatment. To date, there are no curative therapies, although various symptomatic treatment options exist. The present protocol for the management of CI has been drawn up in accordance with the recommendations published in 2012 by the French National Authority for Health, based on a literature review, with the help and validation of members of the French network for rare skin diseases (FIMARAD). It provides a summary of evidence and expert-based recommendations and is intended to help clinicians with the management of these rare and often complex diseases.
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Affiliation(s)
- M Severino-Freire
- University Hospital Center of Toulouse, Reference Centre for Rare Skin Diseases, Department of Dermatology, Larrey Hospital, 24, Chemin de Pouvourville, TSA 30030 Toulouse Cedex 9, France
| | - C Granier Tournier
- University Hospital Center of Toulouse, Reference Centre for Rare Skin Diseases, Department of Dermatology, Larrey Hospital, 24, Chemin de Pouvourville, TSA 30030 Toulouse Cedex 9, France
| | - C Chiaverini
- University Hospital Center of Nice, Department of Dermatology, Archet 2 Hospital, CS 23079, 06202 Nice Cedex 3, France
| | - A Audouze
- Association Ichtyose France, 37 rue du Golf, 03700 Bellerive sur Allier, France
| | - F Morice-Picard
- Department of Dermatology, University Hospital Center of Bordeaux - Hôpital Saint André, 1 Rue Jean Burguet, 33075 Bordeaux Cedex, France
| | - H Texier
- University Hospital Center of Toulouse, Reference Centre for Rare Skin Diseases, Department of Dermatology, Larrey Hospital, 24, Chemin de Pouvourville, TSA 30030 Toulouse Cedex 9, France
| | - I Dreyfus
- University Hospital Center of Toulouse, Reference Centre for Rare Skin Diseases, Department of Dermatology, Larrey Hospital, 24, Chemin de Pouvourville, TSA 30030 Toulouse Cedex 9, France
| | - A-C Bing-Lecointe
- Hospital Annecy-Genevois site Annecy, 1 Avenue De L'hôpital, 74370 Annecy, France
| | - S Mallet
- Department of Dermatology, University Hospital Center of Marseille, 264 rue Saint-Pierre, 13005 Marseille, France
| | - C Bodemer
- Department of Dermatology, Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Hôpital Necker-Enfants Malades, AP-HP, 149 Rue de Sèvres, 75743 Paris cedex 15, France
| | - J Fischer
- Institute of Human Genetics, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Breisacher Straße 153, 79110 Freiburg, Germany
| | - N Jonca
- University Hospital Center of Toulouse, Hôpital Purpan, Cell Biology and Cytology Laboratory, Institut Fédératif de Biologie, Toulouse F-31300, France
| | - J Mazereeuw-Hautier
- University Hospital Center of Toulouse, Reference Centre for Rare Skin Diseases, Department of Dermatology, Larrey Hospital, 24, Chemin de Pouvourville, TSA 30030 Toulouse Cedex 9, France.
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Chulpanova DS, Shaimardanova AA, Ponomarev AS, Elsheikh S, Rizvanov AA, Solovyeva VV. Current Strategies for the Gene Therapy of Autosomal Recessive Congenital Ichthyosis and Other Types of Inherited Ichthyosis. Int J Mol Sci 2022; 23:2506. [PMID: 35269649 PMCID: PMC8910354 DOI: 10.3390/ijms23052506] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2022] [Revised: 02/18/2022] [Accepted: 02/22/2022] [Indexed: 01/27/2023] Open
Abstract
Mutations in genes such as transglutaminase-1 (TGM1), which are responsible for the formation and normal functioning of a lipid barrier, lead to the development of autosomal recessive congenital ichthyosis (ARCI). ARCIs are characterized by varying degrees of hyperkeratosis and the presence of scales on the body surface since birth. The quality of life of patients is often significantly affected, and in order to alleviate the manifestations of the disease, symptomatic therapy with moisturizers, keratolytics, retinoids and other cosmetic substances is often used to improve the condition of the patients' skin. Graft transplantation is commonly used to correct defects of the eye. However, these approaches offer symptomatic treatment that does not restore the lost protein function or provide a long-term skin barrier. Gene and cell therapies are evolving as promising therapy for ARCIs that can correct the functional activity of altered proteins. However, these approaches are still at an early stage of development. This review discusses current studies of gene and cell therapy approaches for various types of ichthyosis and their further prospects for patient treatment.
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Affiliation(s)
- Daria S. Chulpanova
- Institute of Fundamental Medicine and Biology, Kazan Federal University, 420008 Kazan, Russia; (D.S.C.); (A.A.S.); (A.S.P.); (A.A.R.)
| | - Alisa A. Shaimardanova
- Institute of Fundamental Medicine and Biology, Kazan Federal University, 420008 Kazan, Russia; (D.S.C.); (A.A.S.); (A.S.P.); (A.A.R.)
| | - Aleksei S. Ponomarev
- Institute of Fundamental Medicine and Biology, Kazan Federal University, 420008 Kazan, Russia; (D.S.C.); (A.A.S.); (A.S.P.); (A.A.R.)
| | - Somaia Elsheikh
- Division of Cancer and Stem Cell, University of Nottingham, Nottingham LE12 5RD, UK;
| | - Albert A. Rizvanov
- Institute of Fundamental Medicine and Biology, Kazan Federal University, 420008 Kazan, Russia; (D.S.C.); (A.A.S.); (A.S.P.); (A.A.R.)
| | - Valeriya V. Solovyeva
- Institute of Fundamental Medicine and Biology, Kazan Federal University, 420008 Kazan, Russia; (D.S.C.); (A.A.S.); (A.S.P.); (A.A.R.)
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Dorf ILH, Lunen MS, Koppelhus U. Effect of topical treatment with 7.5% urea in Ichthyosis Vulgaris: A randomized, controlled, double blinded, split body study evaluating the effect of urea cream compared to the vehicle (moisturizing) cream. SKIN HEALTH AND DISEASE 2021; 1:e65. [PMID: 35663767 PMCID: PMC9060062 DOI: 10.1002/ski2.65] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/01/2021] [Revised: 08/07/2021] [Accepted: 08/12/2021] [Indexed: 11/19/2022]
Abstract
Background Ichthyosis Vulgaris (IV) is a common genetic skin disease, characterized by dry, scaling skin and itch. Urea cream has been a central part of IV treatment for decades, but only few studies have evaluated the effect of urea compared to basic moisturizers. Objective To evaluate the treatment effect of 7.5% urea cream compared to a basic moisturizer in patients with IV. Methods Participants (n = 14) were randomized to apply the 7.5% urea cream on one body half and a basic moisturizer on the other during a study period of 4 weeks. Measuring points on participants arms and legs were evaluated at baseline and at endpoint with a patient questionnaire visual assessment scale (VAS), a clinical scoring, and electronic skin hydration analysis to assess the treatment effects. Results On the arms, no significant differences between the two treatments were found. On the legs, however, the urea treated areas had a significantly higher decrease in SRRC score (0.7 points [95% CI: 1.1–0.3, p < 0.005]) and increase in hydration (32.1 μS [95% CI: 10.9‐53.2, p < 0.006]). Conclusion Skin hydration improved significantly with both urea and moisturising treatment. On the legs, with most keratinization, urea was superior. Trial registry: https://clinicaltrials.gov/ct2/show/NCT02978209?cond=Ichthyosis+Vulgaris&draw=1&rank=2.
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Affiliation(s)
- I. L. H. Dorf
- Department of Dermatology Aarhus University Hospital Aarhus Denmark
| | - M. S. Lunen
- Department of Intern medicin Regionshospitalet Silkeborg Silkeborg Denmark
| | - U. Koppelhus
- Department of Dermatology Aarhus Universitetshospital Skejby Aarhus Denmark
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Piquero-Casals J, Morgado-Carrasco D, Granger C, Trullàs C, Jesús-Silva A, Krutmann J. Urea in Dermatology: A Review of its Emollient, Moisturizing, Keratolytic, Skin Barrier Enhancing and Antimicrobial Properties. Dermatol Ther (Heidelb) 2021; 11:1905-1915. [PMID: 34596890 PMCID: PMC8611129 DOI: 10.1007/s13555-021-00611-y] [Citation(s) in RCA: 34] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2021] [Indexed: 11/29/2022] Open
Abstract
Urea is a hygroscopic molecule (capable of absorbing water) present in the epidermis as a component of the natural moisturizing factor (NMF) and is essential for the adequate hydration and integrity of the stratum corneum. Urea improves skin barrier function including antimicrobial defense by regulating gene expression in keratinocytes relevant for their differentiation and antimicrobial peptide production. It also plays a fundamental role in regulating keratinocyte proliferation. One of the first uses of urea in modern medicine was the topical treatment of wounds due to its proteolytic and antibacterial properties. At present, urea is widely used in dermatology to improve skin barrier function and as one of the most common moisturizers and keratolytic agents. Urea-containing formulations are available in diverse formulations and concentrations. Multiple clinical trials on the use of urea-containing formulations have shown significant clinical improvement in many of the dermatosis presenting with scaly and dry skin such as atopic dermatitis, ichthyosis, xerosis, seborrheic dermatitis and psoriasis, among others. Furthermore, urea can increase skin penetration and optimize the action of topical drugs. Urea-based products are well tolerated; their side effects are mild and are more frequent at high concentration. Here, we present a review of the use of urea in dermatology, discussing its mechanism of action, safety profile and frequent indications.
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Affiliation(s)
| | - Daniel Morgado-Carrasco
- Dermatology Department, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
| | | | | | | | - Jean Krutmann
- IUF-Leibniz Research Institute for Environmental Medicine, Dusseldorf, Germany.,Medical Faculty, Heinrich Heine University, Düsseldorf, Germany
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Annunziata MC, Cacciapuoti S, Cosentino C, Fabbrocini G. Urea-containing topical formulations. Int J Clin Pract 2020; 74 Suppl 187:e13660. [PMID: 33249709 DOI: 10.1111/ijcp.13660] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2020] [Accepted: 08/06/2020] [Indexed: 12/01/2022] Open
Abstract
Urea is a well-known moisturiser and keratolytic topical agent. As it is widely used in dermatology, several formulations at different concentrations have been marketed: lotions, creams, foams, ointments, gels and lacquers. Availability of different vehicles and concentration may vary in different countries, but in general products at low, medium and high urea concentration are accessible worldwide. The proper formulation should be chosen according to the disorder to treat, its severity, body areas involved and patients' preference.
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Affiliation(s)
- Maria Carmela Annunziata
- Department of Clinical Medicine and Surgery, Section of Dermatology, University of Naples Federico II, Naples, Italy
| | - Sara Cacciapuoti
- Department of Clinical Medicine and Surgery, Section of Dermatology, University of Naples Federico II, Naples, Italy
| | | | - Gabriella Fabbrocini
- Department of Clinical Medicine and Surgery, Section of Dermatology, University of Naples Federico II, Naples, Italy
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6
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Lacarrubba F, Nasca MR, Puglisi DF, Micali G. Clinical evidences of urea at low concentration. Int J Clin Pract 2020; 74 Suppl 187:e13626. [PMID: 33249706 DOI: 10.1111/ijcp.13626] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2020] [Accepted: 07/15/2020] [Indexed: 11/28/2022] Open
Abstract
Urea is a hygroscopic molecule that, because of its moisturising properties, is topically used for the treatment of skin dryness at concentrations ranging from 2% to 12% in different formulations. Based on existing literature, low-concentration urea-containing products are effective in the treatment and/or prevention of xerosis in some skin disorders such as ichthyosis, atopic dermatitis and psoriasis, or unrelated to specific skin diseases. Generally, urea formulations at low concentration are well-tolerated and suited for the treatment of large skin areas, once or twice daily, even for a long period of time. At low concentrations stinging and burning sensation is rare and transient, whit no reported sensitisation despite its widespread use.
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7
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Cortés H, Del Prado-Audelo ML, Urbán-Morlán Z, Alcalá-Alcalá S, González-Torres M, Reyes-Hernández OD, González-Del Carmen M, Leyva-Gómez G. Pharmacological treatments for cutaneous manifestations of inherited ichthyoses. Arch Dermatol Res 2019; 312:237-248. [DOI: 10.1007/s00403-019-01994-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2019] [Revised: 07/26/2019] [Accepted: 10/03/2019] [Indexed: 12/11/2022]
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8
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Mazereeuw-Hautier J, Vahlquist A, Traupe H, Bygum A, Amaro C, Aldwin M, Audouze A, Bodemer C, Bourrat E, Diociaiuti A, Dolenc-Voljc M, Dreyfus I, El Hachem M, Fischer J, Gånemo A, Gouveia C, Gruber R, Hadj-Rabia S, Hohl D, Jonca N, Ezzedine K, Maier D, Malhotra R, Rodriguez M, Ott H, Paige DG, Pietrzak A, Poot F, Schmuth M, Sitek JC, Steijlen P, Wehr G, Moreen M, O'Toole EA, Oji V, Hernandez-Martin A. Management of congenital ichthyoses: European guidelines of care, part one. Br J Dermatol 2018; 180:272-281. [PMID: 30216406 DOI: 10.1111/bjd.17203] [Citation(s) in RCA: 59] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/10/2018] [Indexed: 12/21/2022]
Abstract
These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016 and a consensus on the discussions. They summarize evidence and expert-based recommendations and are intended to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part one, covering topical therapies, systemic therapies, psychosocial management, communicating the diagnosis and genetic counselling.
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Affiliation(s)
- J Mazereeuw-Hautier
- Reference Centre for Rare Skin Diseases, Dermatology Department, Larrey Hospital, Toulouse, France
| | - A Vahlquist
- Department of Medical Sciences, Uppsala University, Uppsala, Sweden
| | - H Traupe
- Department of Dermatology, University Hospital of Münster, Von-Esmarch-Straße 58,, D-48149, Münster, Germany
| | - A Bygum
- Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark
| | - C Amaro
- Hospital de Egas Moniz, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal
| | - M Aldwin
- Ichthyosis Support Group, PO Box 1242, Yateley, GU47 7FL, U.K
| | - A Audouze
- Association Ichtyose France, Bellerive sur Allier, France
| | - C Bodemer
- Department of Dermatology, Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Paris, France.,Institut Imagine, Université Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris
| | - E Bourrat
- Department of Dermatology, Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Paris, France
| | - A Diociaiuti
- Dermatology Division, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy
| | - M Dolenc-Voljc
- Department of Dermatovenereology, University Medical Centre Ljubljana, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - I Dreyfus
- Reference Centre for Rare Skin Diseases, Dermatology Department, Larrey Hospital, Toulouse, France
| | - M El Hachem
- Dermatology Division, Bambino Gesù Children's Hospital-IRCCS, Rome, Italy
| | - J Fischer
- Institute of Human Genetics, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany
| | - A Gånemo
- Department of Dermatology, Institute of Clinical Research in Malmö, Skåne University Hospital, Lund University, Malmö, Sweden
| | - C Gouveia
- Department of Dermatology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - R Gruber
- Department of Dermatology, Venereology and Allergology, Medical University of Innsbruck, Innsbruck, Austria
| | - S Hadj-Rabia
- Department of Dermatology, Reference Center for Genodermatoses and Rare Skin Diseases (MAGEC), Paris, France.,Institut Imagine, Université Descartes, Sorbonne Paris Cité, Hôpital Necker-Enfants Malades, Paris
| | - D Hohl
- Department of Dermatology, Hôpital de Beaumont, Lausanne, Switzerland
| | - N Jonca
- Epithelial Differentiation and Rheumatoid Autoimmunity Unit (UDEAR), UMR 1056 Inserm - Toulouse 3 University, Purpan Hospital, Toulouse, France
| | - K Ezzedine
- Department of Dermatology, Hôpital Henri Mondor, EA EpiDerm, UPEC-Université Paris-Est Créteil, 94010, Créteil, France
| | - D Maier
- Dermatology Department, Iuliu Haţieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - R Malhotra
- Corneoplastic Unit, Queen Victoria Hospital NHS Trust, East Grinstead, U.K
| | - M Rodriguez
- Department of Ear, Nose and Throat, Hospital Universitario Son Espases, Palma de Mallorca, Spain
| | - H Ott
- Division of Pediatric Dermatology and Allergology, Auf Der Bult Children's Hospital, Hanover, Germany
| | - D G Paige
- Department of Dermatology, Royal London Hospital, Barts Health NHS Trust, London, E1 1BB, U.K
| | - A Pietrzak
- Department of Dermatology, Venereology and Paediatric Dermatology, Medical University of Lublin, Lublin, Poland
| | - F Poot
- ULB-Erasme Hospital, Department of Dermatology, Brussels, Belgium
| | - M Schmuth
- Department of Dermatology, Venereology and Allergology, Medical University of Innsbruck, Innsbruck, Austria
| | - J C Sitek
- Department of Dermatology and Centre for Rare Disorders, Oslo University Hospital, Oslo, Norway
| | - P Steijlen
- Department of Dermatology, Maastricht University Medical Centre, GROW Research School for Oncology and Developmental Biology, Maastricht, the Netherlands
| | - G Wehr
- Selbsthilfe Ichthyose, Kürten, Germany
| | - M Moreen
- Department of Dermatology, University Hospitals Leuven, Leuven, Belgium.,Department of Microbiology and Immunology, KU Leuven, Belgium
| | - E A O'Toole
- Centre for Cell Biology and Cutaneous Research, Blizard Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, U.K
| | - V Oji
- Department of Dermatology, University Hospital of Münster, Von-Esmarch-Straße 58,, D-48149, Münster, Germany.,Hautarztpraxis am Buddenturm, Rudolf-von-Langen-Straße 55, D-48147, Münster, Germany
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Parker JC, Scharfbillig RW, Jones S. Effectiveness of Two Moisturizers in the Treatment of Foot Xerosis A Randomized Clinical Trial. J Am Podiatr Med Assoc 2018; 108:458-465. [PMID: 30742522 DOI: 10.7547/16-119] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
BACKGROUND: Xerosis (dryness) of the foot is commonly encountered in clinical care and can lead to discomfort, pain, and predisposition to infection. Many moisturizing products are available, with little definitive research to recommend any particular formulation. METHODS: We compared two commonly prescribed moisturizing products from different ends of the price spectrum (sorbolene and 25% urea cream) for their effectiveness in reducing xerosis signs using the Specified Symptom Sum Score. A randomized clinical trial of parallel design was conducted over 28 days (February-May 2015) on 41 participants with simple xerosis. Participants, therapists, assessors, and data entry personnel were blinded to treatment, and allocation was determined via a randomization table. RESULTS: Thirty-four participants completed the study (19 urea and 15 sorbolene), with one reporting minor adverse effects. There were statistically significant improvements in both groups after 28 days. Mean differences between pre and post scores were 3.50 (95% confidence interval [CI], 2.80 to 4.20) for the urea group and 2.90 (95% CI, 2.00 to 3.80) for the sorbolene group. There was a slightly lower mean posttreatment score in the urea group (1.16; 95% CI, 0.67 to 1.64) than in the sorbolene group (1.80; 95% CI, 1.25 to 2.35), but this difference was not significant ( P ≤ .09). Effect size of difference was -0.48 (95% CI, -1.16 to 0.22). CONCLUSIONS: In this study, there was no difference between using sorbolene or 25% urea cream to treat symptoms of foot xerosis. A recommendation, therefore, cannot be made based on efficacy alone; however, sorbolene treatments are invariably cheaper than urea-based ones.
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Affiliation(s)
- Justin C. Parker
- Department of Podiatry, School of Health Sciences, University of South Australia, Adelaide, Australia
| | - Rolf W. Scharfbillig
- Department of Podiatry, School of Health Sciences, University of South Australia, Adelaide, Australia
| | - Sara Jones
- Department of Health Sciences, University of South Australia, Adelaide, Australia
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10
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Vahlquist A, Fischer J, Törmä H. Inherited Nonsyndromic Ichthyoses: An Update on Pathophysiology, Diagnosis and Treatment. Am J Clin Dermatol 2018; 19:51-66. [PMID: 28815464 PMCID: PMC5797567 DOI: 10.1007/s40257-017-0313-x] [Citation(s) in RCA: 113] [Impact Index Per Article: 16.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Hereditary ichthyoses are due to mutations on one or both alleles of more than 30 different genes, mainly expressed in the upper epidermis. Syndromic as well as nonsyndromic forms of ichthyosis exist. Irrespective of etiology, virtually all types of ichthyosis exhibit a defective epidermal barrier that constitutes the driving force for hyperkeratosis, skin scaling, and inflammation. In nonsyndromic forms, these features are most evident in severe autosomal recessive congenital ichthyosis (ARCI) and epidermolytic ichthyosis, but to some extent also occur in the common type of non-congenital ichthyosis. A correct diagnosis of ichthyosis-essential not only for genetic counseling but also for adequate patient information about prognosis and therapeutic options-is becoming increasingly feasible thanks to recent progress in genetic knowledge and DNA sequencing methods. This paper reviews the most important aspects of nonsyndromic ichthyoses, focusing on new knowledge about the pathophysiology of the disorders, which will hopefully lead to novel ideas about therapy.
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Affiliation(s)
- Anders Vahlquist
- Department of Medical Sciences, Dermatology, Uppsala University, Uppsala, Sweden
| | - Judith Fischer
- Institute of Human Genetics, University Medical Centre, Freiburg, Germany
| | - Hans Törmä
- Department of Medical Sciences, Dermatology, Uppsala University, Uppsala, Sweden.
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11
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Lindh JD, Bradley M. Clinical Effectiveness of Moisturizers in Atopic Dermatitis and Related Disorders: A Systematic Review. Am J Clin Dermatol 2015; 16:341-59. [PMID: 26267423 DOI: 10.1007/s40257-015-0146-4] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
BACKGROUND Moisturizers are widely used for atopic dermatitis (AD) and related conditions, but available evidence of their effectiveness has not been reviewed in a systematic fashion. OBJECTIVES Our objective was to investigate the effectiveness of emollients, as a group and individually, in the treatment of AD and related conditions, by means of a systematic review. DATA SOURCES Studies indexed in MEDLINE and/or Embase before 16 January 2015. STUDY ELIGIBILITY CRITERIA Controlled clinical studies comparing the clinical effect of a moisturizer against its vehicle, another moisturizer, or no treatment were eligible. For the outcomes transepidermal water loss (TEWL) and stratum corneum hydration, uncontrolled before-after designs were also eligible. PARTICIPANTS Participants were patients with AD, irritant hand dermatitis, and/or ichthyosis vulgaris. RESULTS Out of the 595 publications initially identified, 45 (48 studies, 3262 patients) were eligible for inclusion. A vast majority of studies indicate that moisturizers have beneficial effects on clinical symptoms [SCORAD (SCORing Atopic Dermatitis) reductions ranging from 0 to 2.7 points], TEWL (range 0 to -12.2 g/m(2)h) and stratum corneum hydration (range +8 to +100%). Direct comparisons between individual moisturizers are still scarce, but the clinical effect appears to be much more well-documented for urea and glycerin than, for example, propylene glycol, lactate, ceramide, and aluminum chlorohydrate. Compared with urea studies, glycerin studies were more often associated with a high risk of bias. LIMITATIONS Due to differences in study designs and outcome measures, a quantitative meta-analytic approach was not deemed feasible, and formal indicators of publication bias such as funnel plots could not be used. However, a large number of moderately sized studies with positive outcomes could be compatible with selective publishing of favourable results. CONCLUSIONS The clinical effect of moisturizers is well-documented. Urea-based preparations may be preferable as a first-line treatment, but there is an unmet need for well-powered comparisons between individual moisturizers.
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Affiliation(s)
- Jonatan D Lindh
- Division of Clinical Pharmacology, Department of Laboratory Medicine, Karolinska Institutet, Stockholm, Sweden.
| | - Maria Bradley
- Dermatology and Venereology Unit, Department of Medicine Solna, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
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12
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Udompataikul M. New innovation of moisturizers containing non-steroidal anti-inflammatory agents for atopic dermatitis. World J Dermatol 2015; 4:108-113. [DOI: 10.5314/wjd.v4.i2.108] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2014] [Revised: 10/29/2014] [Accepted: 01/19/2015] [Indexed: 02/06/2023] Open
Abstract
Atopic dermatitis is a chronic, relapsing and extremely pruritic eczematous disease which commonly affects children. The standard management consists of a combination of anti-inflammatory drugs in adjunctive with skin care management particular moisturizer application. A concern for the side effects associated with long term use of corticosteroids has also been considered. There has been an emerging interest in moisturizer containing non-steroidal anti-inflammatory agents such as herbal extracts, vitamins, mineral and lipids. The in vitro and the in vivo studies of each agent were reviewed. The clinical study on the efficacy of moisturizers containing these agents were also demonstrated including the author’s studies and clinical experience. These moisturizers might be considered as an alternative treatment in acute flare of mild to moderate atopic dermatitis.
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