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Dowling-Degos disease

Disease definition

A rare, genetic, hyperpigmentation of the skin disease characterized by adulthood-onset of reticular, reddish-brown to dark-brown, macular and/or comedone-like, hyperkeratotic papules with hypopigmented macules, predominantly affecting flexural areas and, on occasion, progressing to involve trunk and acral regions. Histologically, epidermal acanthosis, thin, branch-like, rete ridges, and a tendency for acantholysis and pigmentary incontinence is observed.

ORPHA:79145

Classification level: Disorder
  • Synonym(s):
    • Reticular pigment anomaly of flexures
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Adult
  • ICD-10: L81.8
  • OMIM: 179850  615327  615674  615696
  • UMLS: C3714534
  • MeSH: -
  • GARD: 9775
  • MedDRA: 10068651
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