title: Dowling-Degos disease: classic clinical and histopathological presentation.
abstract: Dowling-Degos disease (DDD) is a rare genetic disease of the skin (reticulate pigmented anomaly), clinically characterized by flexural brown pigmented reticulate macules, comedo-like papules on the back, neck and pitted perioral or facial scars. We present the case of a 51 year-old man with macrocomedo-like lesions, pitted scars, cysts, hyperpigmented macules in his back, chest, axillae, neck, groin and face. The patient reported having two children, three brothers and a father with a similar condition. The histopathology of the skin biopsies was very characteristic of Dowling-Degos disease, showing dilated follicular, fingerlike projections called rete ridges (dermal pegs), with thinning of the suprapapillary plates, resulting in an "antler-like" pattern and increased pigmentation of the basal layer.
authors.affiliation: Hospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Brasil. carolczim@gmail.com
authors.forename: Carolina Cotta
authors.initials: CC
authors.lastname: Zimmermann
authors.forename: Deborah
authors.initials: D
authors.lastname: Sforza
authors.forename: Priscila Marques de
authors.initials: PM
authors.lastname: Macedo
authors.forename: Luna
authors.initials: L
authors.lastname: Azulay-Abulafia
authors.forename: Maria de Fatima G S
authors.initials: Mde F
authors.lastname: Alves
authors.forename: Sueli Coelho da S
authors.initials: SC
authors.lastname: Carneiro
mesh_terms: D001706:Biopsy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010859:Pigmentation Disorders / Q000235:genetics / Q000473:pathology*; D012867:Skin / Q000473:pathology*; D012873:Skin Diseases, Genetic / Q000473:pathology*