Pemphigus vulgaris (PV) is an anti-cadherin autoimmune disease, characterized by the production of anti-desmoglein 3 (and to a lesser extent anti-desmoglein 1) antibodies, producing acanthosis, typically suprabasal, which clinically translates into flaccid blisters and erosions, generally starting on mucous membranes, with subsequent appearance of skin lesions. Only about 25 cases of PV of exclusively cutaneous location have been described in the literature. Treatment with potent topical steroids is usually sufficient to control the symptoms.
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