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At a glance

A rare, blistering autoimmune life-threatening disease that affects the skin and mucous membranes. Patients have circulating antibodies to an intercellular cement substance of the basal membrane.

Pemphigus Vulgaris: Enoral ulcerations in young man with pemphigus vulgaris.

Synonyms

Pemphigus Vulgaris; Pemphigus Foliaceus; Hailey-Hailey Disease; Senear-Usher Syndrome; Pemphigus Erythematosus; Benign Familial Pemphigus; Pemphigus Herpetiformis.

Incidence

Estimated 1 to 5:100,000 in the United States. It affects males and females in equal numbers and is most common in middle-aged and elderly people. Few cases have been reported in children. All ethnic groups and races can be affected; however, it is believed to be more common in Jewish or Mediterranean (Iraqi, Iranian, Indian) descent.

Classification

Large family of rare blistering disease.

  • Pemphigus Vulgaris: The most common familial pemphigus vulgaris. Occurs predominantly in Jewish or Mediterranean peoples. It usually appears as painful nonhealing oral ulcers, which may be the only evidence of the disease for weeks or months. The typical lesions are small, flaccid bullae arising from normal skin; they rupture rapidly, leaving nonhealing painful erosions. Patients often complain of burning and pain. Lesions may be present in the esophagus and nasal cavity. The blister is intraepidermal; autoantibodies directed to desmoglein 3 (a keratinocyte surface antigen) are present. Untreated, the disease normally progresses to loss of most of the epidermis and death. It can appear antenatally from women who have an active pemphigus or who are in remission. The outcome of the fetus is very poor, including demise.

  • Pemphigus Vegetans: A variant of Pemphigus Vulgaris. The blisters are fast-growing and hypertrophic lesions in the inguinal and axillary areas.

  • Pemphigus Foliaceus: This is extremely rare, and presents as small crusted lesions on the scalp, face, chest, and back. The site of cleavage is high in the epidermis. Autoantibodies directed to desmoglein 1 are present. It often resembles seborrheic dermatitis.

  • Pemphigus Herpetiformis: A subtle form of pemphigus with its own characteristic blisters. However, blisters that form during a relapse may resemble those of Pemphigus Foliaceus.

  • Paraneoplastic Pemphigus: Autoimmune syndrome associated with neoplasms of various origins. Characterized by painful, extensive mucosal erosions and lichenoid papules of the extremities. Mostly associated with lymphoproliferative disorders such as chronic lymphocytic leukemia, plasmocytoma, and ☞Castleman’s Disease and B Cell Lymphoma.

  • Hailey-Hailey Disease (Benign Familial Pemphigus; Benign Chronic Pemphigus): This is an autosomal dominant disease. The recurrent eruption of bullae and vesicles involves predominantly the neck, groin, and axillary areas. The acantholysis is suprabasal. There could be some overlap between Darier and Hailey-Hailey disease.

  • Pemphigus Erythematosus (Senear-Usher Syndrome): Pemphigus is an autoimmune bullous skin disease with acantholysis in the epidermis. The cutaneous lesions often consist of well-marginated plaques that are erythematous, scaly, hyperkeratotic, and crusted. The lesions have a seborrheic distribution ...

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