Type 1 segmental Darier’s disease in a 43-year-old woman
Keywords:
acantholysis, Darier’s disease, mosaicism, retinoidsAbstract
Segmental Darier’s disease is a rare clinical variant characterized by a linear distribution of keratotic papules following Blaschko´s lines. We report the case of a 43-year-old woman who presented with a 4-month history of a lesion on her neck displaying a linear arrangement; histologically, epidermis with orthokeratotic hyperkeratosis and multifocal parakeratosis, intraepidermal clefts with acantholytic cells, and corps ronds in the granular layer, were seen. Treatment with vitamin A 100,000 IU per day, for two months, and 0.1% retinoic acid cream, once a day, was indicated, with overall improvement.
This is a model case of type 1 segmental Darier’s disease with a successful response to the treatment; it is also representative of that group of entities that follow Blaschko´s lines, which, as a rule, are related to a mosaicism of the involved skin.
Author Biographies
Rolando Rojas
Médico, residente de Dermatología, Facultad de Medicina, Universidad Nacional de Colombia, Bogotá, D.C., Colombia; Centro Dermatológico Federico Lleras Acosta, E.S.E., Bogotá, D.C., Colombia
Raquel Eraso
Médica dermatóloga, Centro Dermatológico Federico Lleras Acosta, E.S.E., Bogotá, D.C., Colombia
Héctor Castellanos
Médico dermatólogo; profesor asociado de Dermatología, Facultad de Medicina, Universidad Nacional de Colombia, Bogotá, D.C., Colombia; Centro Dermatológico Federico Lleras Acosta, E.S.E., Bogotá, D.C., Colombia
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