Co-existence of leukoderma with features of dowling-degos disease: Reticulate acropigmentation of kitamura spectrum in five unrelated patients

G. G. Lestringant, I. Masouyé, P. M. Frossard, E. Adeghate, I. H. Galadari

Research output: Contribution to journalArticlepeer-review

31 Citations (Scopus)

Abstract

Background: The spectrum of Dowling-Degos disease-reticulate acropigmentation of Kitamura (DDD-RAK) is a group of rare autosomal dominant disorders that have in common a unique histological picture of hyperpigmented digitate epidermal ‘downgrowths’. Patients with the DDD-RAK spectrum may show hyperpigmented macules and papules, facial pits, breaks in dermatoglyphics and epidermoid cysts. Observations: We examined 5 unrelated patients, 3 females and 2 males (age range 22–35 years), who presented with clinical and histological features of the DDD-RAK spectrum. In addition, the patients presented with hypo- or depigmented macules and papules. Histopathology of the lesions revealed features that were identical to DDD-RAK; there were, however, diminution or absence of pigmentation. Family histories for pigmented lesions and leukoderma were positive in all patients and consistent with autosomal dominant modes of inheritance. Conclusion: These 5 cases, together with isolated reports in the literature of achromic lesions with histological features of DDD-RAK, point to the hypothesis that achromic macules and papules may be a feature of the DDD-RAK spectrum.

Original languageEnglish
Pages (from-to)337-343
Number of pages7
JournalDermatology
Volume195
Issue number4
DOIs
Publication statusPublished - Jan 1997

Keywords

  • Dowling-Degos Disease (DDD)
  • Leukoderma
  • Reticulate acropigmentation of Kitamura (RAK)
  • United Arab Emirates

ASJC Scopus subject areas

  • Dermatology

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