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Revista argentina de dermatología

On-line version ISSN 1851-300X

Abstract

AVILA, Y Velázquez; PEREZ, O Peña  and  YABOR, VJ Álvarez. Hailey-Hailey diseases, a family diagnosed starting from a case propositus. Rev. argent. dermatol. [online]. 2020, vol.101, n.4, pp.11-20. ISSN 1851-300X.

Introduction:

The Hailey-Hailey diseases,also known asbenign familial pemphigus, is an disease of very low appearance frequency,although it is a genodermatoses, the clinical manifestations are manifested in the adolescence or early adulthood.

Objective:

To deepen in the elements that allows the early diagnosis of Hailey-Hailey diseases.

Case presentation:

In Las Tunas, oriental county of Cuba, is assisted in multidisciplinary specialized consultation of genodermatoses, a 50-year-old woman who presented lesions erytmematous, vesiculous and erosive, located in areas of pleats that had appeared from the adolescence, being treated in several occasions like a superficial mycosis or dermatitis. She is carried out study histopathologyc that verified the diagnosis of Hailey-Hailey diseases. The other members of the family were studied affected with similar injure dermatological and the family diagnosis is corroborated. The patient was treated with steroids in dose antinflammatory, vitamintherapy and topical therapeutic consistentin use of antiseptic natural foments and cream antinflammatory of aloe, with good answer to the therapy.

Conclusions:

The case is presented because the Hailey-Hailey diseases areuncommon; the manifestations begin in the adolescence, being able to make a mistake with other dermatomes.In the investigation the diagnosis of the other members of the family was determined affected starting from the case propositus.

Keywords : Medical Genetics, Diseases Of The Skin; Genodermatoses, Hailey-Hailey Diseases, Benign Familial Pemphigus.

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