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Journal of the American Academy of Dermatology
Volume 43, n° 4
pages 571-594 (octobre 2000)
Doi : 10.1067/mjd.2000.107248
Cicatricial pemphigoid

Thomas E. Fleming, MD, Neil J. Korman, MD, PhD
Department of Dermatology, Case Western Reserve University. Cleveland, Ohio 


Cicatricial pemphigoid (CP) is a heterogeneous group of rare, chronic, subepithelial blistering disorders of the mucous membranes and, occasionally, the skin, which can have serious and rarely fatal consequences. The most common clinical features are desquamative gingivitis, oral erosions, and conjunctival fibrosis. Skin lesions occur less frequently and may present as widespread vesicles and bullae, as in bullous pemphigoid (BP). In some patients, the scarring can be a source of significant morbidity because it can result in odynophagia, strictures of the upper aerodigestive tract, or corneal opacities leading to eventual blindness. This article is a comprehensive review and discusses clinical, pathologic, and pathophysiologic aspects of this group of disorders collectively known as CP. (J Am Acad Dermatol 2000;43:571-91.) Learning objective: At the conclusion of this learning activity, participants should be familiar with the clinical spectrum of CP, the histopathologic and immunopathologic characteristics, the differential diagnosis, the treatment, and the natural history of the disease. Furthermore, this learning activity should facilitate early diagnosis of CP and should promote the idea that the involvement of other specialists, including ophthalmologists, otolaryngologists, gastroenterologists, and oral medicine specialists, as appropriate, will aid in providing these patients with the highest quality of care.

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Abbreviations : BMZ:, BP:, CP:, DIF:, EBA:, IIF:, LABD:, SLE:

 Supported by National Institutes of Health grant R29 AR41008.
 Reprint requests: Neil J. Korman, PhD, MD, Department of Dermatology, University Hospitals of Cleveland, 11100 Euclid Ave, Cleveland, OH 44106. E-mail:
 J Am Acad Dermatol 2000;43:571-91.

© 2000  American Academy of Dermatology, Inc. Published by Elsevier Masson SAS@@#104157@@
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