Orphanet: Reticulate acropigmentation of Kitamura

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Reticulate acropigmentation of Kitamura

Disease definition

A rare, genetic, hyperpigmentation of the skin disease characterized by childhood to adulthood-onset of reticulate, slightly depressed, sharply demarcated, brown, macular skin lesions without hypopigmentation, affecting the dorsa of the hands and feet, and, occasionally, progressing to involve limbs, neck, forehead and/or trunk. Interrupted dermatoglyphics and palmoplantar pits may be additionally observed. Histologically, hyperpigmented lesions show slightly elongated and thinned rete ridges, mild hyperkeratosis without parakeratosis and absence of incontinentia pigmenti.


Classification level: Disorder
  • Synonym(s):
    • RAK
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood, Adolescent, Adult
  • ICD-10: L81.8
  • OMIM: 615537
  • UMLS: C0406811
  • MeSH: -
  • GARD: -
  • MedDRA: -
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