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Pemphigus

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Jean-Claude Bystryn, MD, deceased, former Professor of Dermatology and Director of the Bullous Disease Clinic, Department of Dermatology, NYU School of Medicine, and to Victoria P. Werth, MD, Chairman, International Pemphigus and Pemphigoid Foundation (IPPF) Medical Advisory Board; Professor, Department of Dermatology, University of Pennsylvania; Chief of Dermatology, Philadelphia V.A. Medical Center, for assistance in the preparation of this report.

Synonyms of Pemphigus

  • No synonyms found.

Disorder Subdivisions

  • drug-induced pemphigus
  • endemic pemphigus (fogo selvagem)
  • pemphigus erythematosus (Senear-Usher syndrome)
  • pemphigus foliaceus
  • pemphigus vegetans
  • pemphigus vulgaris

General Discussion

Pemphigus is a general term for a group of rare autoimmune blistering skin disorders. Autoimmune disorders occur when the body's own immune system mistakenly attacks healthy tissue. The symptoms and severity associated with the various forms of pemphigus vary. All forms of pemphigus are characterized by the development of blistering eruptions on the outer layer of the skin (epidermis). In pemphigus vulgaris, lesions also develop on the mucous membranes such as those lining the inside the mouth. Mucous membranes are the thin, moist coverings of many of the body's internal surfaces. If left untreated, pemphigus will usually be fatal. The exact cause of pemphigus is unknown.

The term pemphigus is a general term for a group of related autoimmune blistering skin diseases. The two main types of pemphigus are pemphigus vulgaris and pemphigus foliaceus. Each type has subtypes. Additional disorders are sometimes classified as pemphigus including paraneoplastic pemphigus and pemphigus IgA. Some physicians consider these disorders similar, yet distinct, autoimmune blistering disorders with different causes and clinical, immunological and microscopic tissue (histological) features. Pemphigoid is a general term for a different group of skin disorders. These other disorders are discussed in the related disorders section of this report.

Organizations related to Pemphigus

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