Immunome Research
Open Access
ISSN: 1745-7580
+44-20-4587-4809
ISSN: 1745-7580
+44-20-4587-4809
Department of Dermatology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
Case Report
Pemphigus Herpetiformis with Only Positive Anti-Envoplakin Antibodies: A Case Report
Author(s): Cristina Sousa*, Manuel Campos, Jorge Lopes, Armando Baptista and 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 pemph.. View More»