Pemphigus is a chronic autoimmune blistering disease provoked by autoantibodies reacting with normal constituants of desmosomes, the structures that provide attachment between epidermal cells. It presents clinically with flaccid intraepidermal blisters and erosions of the skin and mucous membranes (Figure 34.8, between pp. 426 and 427). Nikolsky's sign is found.
The histology exhibits detachment of epidermal cells (acantholysis), responsible for intraepidermal blisters located subcorneally (pemphigus foliaceus) or in the lower epidermis (pemphigus vulgaris).
Direct immunofluorescence performed to a perilesional skin biopsy specimen reveals immuno-globulin deposits around keratinocytes in the epidermis in all "spontaneous" cases but in only 50% of drug-induced cases. The presence in the serum of autoantibodies reacting against the epidermis is detected by indirect immunofluores-cence, western-blot or ELISA tests.
In western countries about 10% of cases of pemphigus could be drug-induced. It begins several weeks or months after drug therapy is initiated. It presents as pemphigus foliaceus or as pemphigus vulgaris with mucosal involvement. The main drugs incriminated are d-penicillamine and other drugs containing a thiol radical, like captopril and piroxicam. The remission after drug withdrawal is not always spontaneous, particularly in cases of pemphigus attributed to drugs that do not have a thiol part.
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