Dowling-Degos disease: a review

Int J Dermatol. 2021 Aug;60(8):944-950. doi: 10.1111/ijd.15385. Epub 2020 Dec 23.


Dowling-Degos disease is a rare autosomal dominant genodermatosis. It is characterized by acquired reticulate hyperpigmentation over the flexures, comedone-like follicular papules, and pitted perioral scars that usually develop during adulthood. Mutations in genes affecting melanosome transfer, and melanocyte and keratinocyte differentiation have been implicated in the pathogenesis of this disease. These genes include KRT5, POFUT1, POGLUT1 and, most recently, PSENEN. Dowling-Degos disease can be found in isolation or with other associated findings, most notably hidradenitis suppurativa. This condition belongs to a spectrum of conditions that all result in reticulate hyperpigmentation that at times are hard to distinguish from each other. The most closely linked entity is Galli-Galli, which is clinically indistinguishable from Dowling-Degos disease and can only be distinguished by the presence of acantholysis on microscopy. Unfortunately, Dowling-Degos disease is generally progressive and recalcitrant to treatment.

Publication types

  • Review

MeSH terms

  • Acantholysis / diagnosis
  • Acantholysis / genetics
  • Adult
  • Amyloid Precursor Protein Secretases
  • Fucosyltransferases
  • Glucosyltransferases
  • Humans
  • Hyperpigmentation* / diagnosis
  • Hyperpigmentation* / genetics
  • Keratin-5
  • Membrane Proteins
  • Skin Diseases, Genetic* / diagnosis
  • Skin Diseases, Genetic* / genetics
  • Skin Diseases, Papulosquamous* / diagnosis
  • Skin Diseases, Papulosquamous* / genetics


  • KRT5 protein, human
  • Keratin-5
  • Membrane Proteins
  • PSENEN protein, human
  • Fucosyltransferases
  • Glucosyltransferases
  • POGLUT1 protein, human
  • polypeptide fucosyltransferase
  • Amyloid Precursor Protein Secretases

Supplementary concepts

  • Dowling-Degos Disease