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Pemphigus Herpetiformis with Only Positive Anti-Envoplakin Antibodies: A Case Report

Cristina Sousa, Manuel Campos, Jorge Lopes, Armando Baptista, Ana Oliveira

Pemphigus herpetiformis, first described in 1975, is a rare subtype of autoimmune bullous disease that associates clinical features of dermatitis herpetiformis with immunological features of pemphigus. We present a 48-years-old female patient, with pruritic lesions located mainly in the upper limbs with 6 years of evolution. Physical examination revealed erythematous papules, some excoriated and vesicles of translucent content distributed on her arms, trunk and neck. Histological examination of a skin biopsy revealed the presence of sub epidermal vesicles associated with necrosis of the epidermis and perivascular lymphocytic infiltrate in the papillary and reticular dermis. The immunological study was positive for antibodies to intercellular IgG and anti-envoplakin antibodies. Anti-desmoglein 1 and 3 antibodies were negative. Considering these results, the clinical hypothesis of pemphigus herpetiformis was admitted. The positivity of the anti-envoplaquine antibody is often associated with paraneoplastic pemphigus, so a cancer screening was performed which was negative. Prednisolone and dapsone was introduced with resolution of lesions. To our knowledge this is the first clinical case of pemphigus herpetiformis with positive anti-envoplakin antibodies. This rare and often overlooked entity has a good prognosis, although there are cases reports with progression to pemphigus vulgaris or pemphigus foliaceus.