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Reticulate acropigmentation of Kitamura

ORPHA:178307· ICD-10 L81.8

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.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Adolescent, Adult, Childhood