Pemphigus is a group of rare autoimmune skin disorders characterized by the development of blisters in the outer layer of the skin (epidermis) and mucous membranes (thin moist layers that line the body's internal surfaces). The location and type of blisters vary according to the type of Pemphigus. If left untreated Pemphigus can be a serious illness. Mostly misdiagnosed as Herpes.
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Blisters in the outer layer of the skin are common to all types of Pemphigus. Blisters develop due to the destruction of the "cement" that holds cells together (epidermal acantholysis) resulting in the separation of cells from one another. Soft (flaccid) blisters generally occur on the neck, scalp, mucous membranes, and/or underarm (axillary) and groin areas (inguinal). Most patients with Pemphigus have deposits of IgG (an immune system antibody that defends against foreign substances) around the blistered areas (in the epidermal cells called keratinocytes). Antiepidermal antibodies directed against skin cells are typically present in the fluid of the blisters. The diagnosis of Pemphigus requires microscopic examination of cells in the blisters as well as detection of the IgG antibodies that characterize this disease.
Pemphigus Vulgaris is the most common form and may begin with isolated blisters on the scalp, and then in the mouth. These may persist for several months and may be followed by blistering of the esophagus, nose, rectum, and/or the membranes that line the inner surfaces of the eyelids (conjunctiva). The blisters are soft; they break easily and heal poorly. Pressure on the borders of blisters causes them to spread. Pressure on normal-looking skin can cause it to blister (Nikolsky sign) in people with Pemphigus Vulgaris. If left untreated, Pemphigus Vulgaris may cause life-threatening complications.
Pemphigus Vegetans is a variation of Pemphigus Vulgaris. The blisters are fast-growing and have large (hypertrophic) lesions that are usually located in the groin (inguinal) and armpit (axillary) areas.
Pemphigus Foliaceus is less severe and a less common form of the disorder. Soft blisters typically occur close to the surface of the skin. When they break, they ooze and become crusty, scaly, and susceptible to infection. Blisters may occur on the scalp, face, upper chest, and back; the mucous membranes are usually not affected. Small, horny plugs attached to the undersurface of the affected skin also may be seen.
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