COEXISTENCE OF LEUKODERMA WITH FEATURES OF DOWLING-DEGOS-DISEASE - RETICULATE ACROPIGMENTATION OF KITAMURA SPECTRUM IN 5 UNRELATED PATIENTS

Background: The spectrum of Dowling-Degos disease-reticulate acropigme ntation of Kitamura (DDD-RAK) is a group of rare autosomal dominant di sorders that have in common a unique histological picture of hyperpigm ented digitate epidermal 'downgrowths'. Patients with the DDD-RAK spec trum may show hyperpigmented macules and papules, facial pits, breaks in dermatoglyphics and epidermoid cysts. Observations: We examined 5 u nrelated patients, 3 females and 2 males (age range 22-35 years), who presented with clinical and histological features of the DDD-RAK spect rum. In addition, the patients presented with hypo-or depigmented macu les 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 isolate d reports in the literature of achromic lesions with histological feat ures of DDD-RAK, point to the hypothesis that achromic macules and pap ules may be a feature of the DDD-RAK spectrum.