Genital Hailey- Hailey disease: a case report

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 th...

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Main Authors: Deeptara Pathak Thapa (Author), Anil Kumar Jha (Author), Sujata Pudasaini (Author), Chandani Kharel (Author), Shristi Shrestha (Author)
Format: Book
Published: Our Dermatology Online, 2013-01-01T00:00:00Z.
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520 |a 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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