Segmental Darier's disease: a presentation of difficult diagnosis* * Work performed at Hospital Universitário Pedro Ernesto da Universidade do Estado do Rio de Janeiro (HUPE – UERJ) – Rio de Janeiro (RJ), Brazil.

Paula Mota Medeiros Natália Ribeiro de Magalhães Alves Jeniffer Muñoz Trujillo Cássia Camarinha da Silva Paula Carolina Pessanha de Faria Roberto Souto da Silva About the authors


Darier's disease is a rare autosomal dominant genodermatosis. It has an estimated prevalence of 1 in 55,000 to 100,000 individuals, regardless of gender. It is characterized by multiple keratotic papules on the seborrheic areas of the trunk, scalp, forehead and flexures, and the clinical picture is worsened by heat, sun exposure, perspiration and mechanical trauma. Histopathology observed loss of epithelial adhesion and abnormal keratinization. About 10% of cases present in the localized form of the disease. We report a case of segmental Darier's Disease Type I and discuss the main characteristics of this condition.

Darier's Disease; Mosaicism; Skin diseases; Skin diseases, genetic

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