A rare, blistering autoimmune disease that affects the skin
and mucous membranes. Patients have circulating antibodies to an
intercellular cement substance of the basal membrane.
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 descent. It occurs
once in 100,000 people in the United States.
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 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
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
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 and are often present in a butterfly pattern over
the malar area.
Most forms of pemphigus are generally
considered to be autoimmune-related. However, Benign Familial Pemphigus (Hailey-Hailey Disease) is inherited
as an autosomal dominant transmission. The gene responsible is chromosome 3 (3q21-q24). Fogo Selvagem
(Brazilian Pemphigus Foliaceus) is an autoimmune blistering disorder transmitted through the bite of blackflies.
Pemphigus may also occur following x-ray exposure or adverse reaction to drugs such as d-penicillamine
Blisters in the outer layer of the skin are
common to all types of pemphigus (epidermal acantholysis). Deposits of IgG
in the epidermal cells are called keratinocytes. Antiepidermal antibodies
directed against skin cells are typically ...