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Reticulate acropigmentation of Kitamura
Other Resources UpToDate PubMed

Reticulate acropigmentation of Kitamura

Contributors: Ailia Ali MD, Susan Burgin MD
Other Resources UpToDate PubMed


Reticulate acropigmentation of Kitamura (RAPK) is a rare autosomal dominantly inherited pigmentary disorder characterized by slightly depressed hyperpigmented reticulated papules on the dorsal hands and feet. Onset is typically at age 5-12 years, and progression with involvement of the distal extremities is seen over ensuing years. The lesions tend to gradually darken over time. They can also darken with exposure to the sun and may be more prominent in the summer. In addition to these features, patients with RAPK also have palmoplantar pits and breaks in the creases of the palms, soles, and fingers.

RAPK was first described in the Japanese population, and it has since been documented in many ethnic groups around the world. RAPK is seen more commonly in females than in males.

RAPK is caused by a mutation in the ADAM10 gene on chromosome 15q21.


L81.8 – Other specified disorders of pigmentation

239133004 – Reticulate acropigmentation of Kitamura

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Differential Diagnosis & Pitfalls

  • Reticulate pigmented anomaly of flexures (DDD)
  • Dyschromatosis symmetrica hereditaria – Hyperpigmented and hypopigmented macules; predominantly males will be affected.
  • Dyschromatosis universalis hereditaria (DUH) – Hyperpigmented and hypopigmented macules diffusely present over trunk; autosomal dominant.
  • Xeroderma pigmentosum
  • Naegeli syndrome (NFJS)
  • Dyskeratosis congenita
  • Dermatopathia pigmentosa reticularis (DPR)
  • Ephelides
  • Familial progressive hyperpigmentation – This will be present at birth or soon after. Hyperpigmented patches of varying sizes occur on skin and mucous membranes.
  • Cole syndrome

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Last Reviewed:08/07/2018
Last Updated:01/23/2022
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Reticulate acropigmentation of Kitamura
A medical illustration showing key findings of Reticulate acropigmentation of Kitamura : Symmetric extremities distribution
Copyright © 2024 VisualDx®. All rights reserved.