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Nail Involvement as the Only Onset Manifestation of Pemphigus Vulgaris

Jose Salvador García-Morillo, Francisco Javier Nieto Garcia, Alberto Ruiz Román and Alejandro Ortiz Prieto

Pemphigus vulgaris (PV) is a blistering autoimmune, chronic and uncommon disease that affects the skin, mucous membranes and skin appendages. The onset of the disease usually consists of mucosal lesions (50%-70%), skin manifestations (10%-15%), or both. Nail involvement usually develops in association with the previously mentioned manifestations, being exceptionally the only affected area at the onset of the disease.

A 52 year old patient with no important medical history was evaluated after two weeks of antibiotic treatment for painful, swollen, erythematous-violaceous, poorly delimited lesions, with subungueal haemorrhage, affecting the nails and periungueal tissue of the first and third right toes and the third left finger; the nail plate presented hyperkeratosis, yellowish discoloration and distal onycholysis. Complementary blood analysis and skin cultures did not reveal positive results. A skin biopsy showed suprabasal acantholytic dermatosis with no inflammatory components, and immunofluorescence manifested IgG intercellular deposits, with no evidence of IgA, IgM, C3 or fibrinogen deposits. Autoimmune serologic studies were positive for anti-intercellular substance antibody (1/80) and negative for anti-basal membrane and anti-nuclear antibodies. With these findings the patient was diagnosed with Pemphigus Vulgaris, being nail lesions the exclusive onset manifestation.