Abstract

CHAUSTRE TORRES, Linda Adori; TORRA BARAJAS, Oscar Leonardo; RUBIANO, Cesar Augusto  and  GUTIERREZ MORENO, Mario Fernando. Atypical presentation of familial benign pemphigus in patient without familiar background. Medicas UIS [online]. 2021, vol.34, n.1, pp.101-106.  Epub Apr 01, 2021. ISSN 0121-0319.  https://doi.org/10.18273/revmed.v34n1-2021010.

Hailey-Hailey disease, also called benign familial pemphigus, corresponds to a debilitating genodermatosis that is transmitted through an autosomal dominant pattern, with a prevalence of around 1 in 50,000 cases. The family history report is present in up to 60% of patients. It is characterized by the presence of chronic and recurrent vesicles, erosions and exulcerations in flexural areas. Treatment can be challenging, because despite management with topical therapies, systemic corticosteroids, systemic immunomodulators, and the use of lasers, no therapy has achieved long-term remission.We present the case of a male patient, middle adult, without any family history, with a history of scaly surface plaques and chronic recurrent hematocostras and atypical clinical presentation given the location of predominant lesions in the upper limbs, with initial suspicion of vulgar psoriasis, with subsequent biopsy and pathology report showing typical PBF histology. Therefore, management with systemic corticosteroids without evidence of adverse reactions and with long-term remission is indicated. MÉD.UIS.2020;34(1):101-6

Keywords : Familial benign pemphigus; Hailey-Hailey disease; Acantholysis; Treatment; Corticosteroids.

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