TY - JOUR
T1 - Co-existence of leukoderma with features of dowling-degos disease
T2 - Reticulate acropigmentation of kitamura spectrum in five unrelated patients
AU - Lestringant, G. G.
AU - Masouyé, I.
AU - Frossard, P. M.
AU - Adeghate, E.
AU - Galadari, I. H.
PY - 1997/1
Y1 - 1997/1
N2 - 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.
AB - 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.
KW - Dowling-Degos Disease (DDD)
KW - Leukoderma
KW - Reticulate acropigmentation of Kitamura (RAK)
KW - United Arab Emirates
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U2 - 10.1159/000245984
DO - 10.1159/000245984
M3 - Article
C2 - 9529553
AN - SCOPUS:0031458102
SN - 1018-8665
VL - 195
SP - 337
EP - 343
JO - Dermatology
JF - Dermatology
IS - 4
ER -