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Acta Med Croatica. 2012 Oct;66 Suppl 1:41-7.

[Pemphigus chronicus vulgaris--case report].

[Article in Croatian]

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Sestre milosrdnice University Hospital Center, University Department of Dermatovenereology, Zagreb, Croatia.


Pemphigus vulgaris is a rare, chronic, autoimmune blistering skin disease of adulthood. In 50 percent of patients, mucous membrane erosions of the oral cavity are the presenting sign. Typically, patients develop flaccid blisters and erosions on the normal appearing skin and mucous membranes. Mucous membranes in other areas may also be involved. The disease is caused by IgG autoantibodies to the desmosomes inducing the loss of cell adhesion between keratinocytes, and subsequent intraepidermal blister formation. Antigens are desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1) transmembrane glycoproteins. Desmoglein 3 is expressed in the lower portion of the epidermis. Alone, it is sufficient to keep mucosal surfaces intact. Desmoglein 1 is expressed more intensely in the superficial layers. Pemphigus vulgaris can be divided into two subgroups, the mucosal type and mucocutaneous type. The characteristic histologic finding is intraepidermal blister. Acantholytic keratinocytes as well as clusters of epidermal cells are seen in the blister cavity. Direct immunofluorescence examination (DIF) of perilesional skin shows a deposit of IgG, rarely IgA, and complement components (C3, C1q,C4) between the epidermal cells. The titers of circulating antibodies measured with indirect immunofluorescence examination (IIF) often correlate with the disease course. There are sporadic cases of pemphigus vulgaris associated with the use of drugs, in particular D-penicillamine and captopril. Pemphigus vulgaris has rarely evolved into pemphigus foliaceus, and vice versa. Very uncommon, pemphigus vulgaris or pemphigus foliaceus has been associated with bullous pemphigoid in the same patient. We present an unusual case of pemphigus vulgaris in an adult female patient.

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