Dowling-Degos disease: classic clinical and histopathological presentation

An Bras Dermatol. 2011 Sep-Oct;86(5):979-82. doi: 10.1590/s0365-05962011000500016.
[Article in English, Portuguese]

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.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Humans
  • Male
  • Middle Aged
  • Pigmentation Disorders / genetics
  • Pigmentation Disorders / pathology*
  • Skin / pathology*
  • Skin Diseases, Genetic / pathology*