Enfermedad de Darier segmentaria de tipo 1 en una mujer de 43 años.

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. [ABSTRACT FROM AUTHOR]