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Genital Hailey- Hailey disease: a case report

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Author(s): Deeptara Pathak Thapa | Anil Kumar Jha | Sujata Pudasaini | Chandani Kharel | Shristi Shrestha

Journal: Our Dermatology Online
ISSN 2081-9390

Volume: 4;
Issue: 1;
Start page: 87;
Date: 2013;
Original page

Keywords: acantholysis | Hailey – Hailey disease | Nepal

ABSTRACT
Hailey – Hailey disease is a rare autosomal dominant acantholytic disorder, previously not reported from Nepal. We report a case of 30 years old female who presented with pruritic hyperkeratotic papules and plaques on vulva, perianal area and inner left thigh for a period of one year. Biopsy from the lesion showed suprabasal acantholysis with loss of intercellular bridges resulting in a dilapidated brick-wall appearance; characteristic of Hailey – Hailey disease. Treatment of this disease till date is far from satisfactory.
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