Dowling-Degos disease
ORPHA:79145· ICD-10 L81.8
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.
- Prevalence
- Unknown
- Inheritance
- Autosomal dominant
- Age of onset
- Adolescent, Adult