Co-existence of leukoderma with features of Dowling-Degos disease: reticulate acropigmentation of Kitamura spectrum in five unrelated patients.
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.


METHODS
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.


CONCLUSIONS
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.
Lestringant
G G
GG
Department of Dermatology, Tawam Hospital, Al Ain, United Arab Emirates. gglest@emirates.net.ae
Masouyé
I
I
Frossard
P M
PM
Adeghate
E
E
Galadari
I H
IH
D000169:Acrodermatitis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D000328:Adult; D001284:Atrophy; D003878:Dermatoglyphics; D004814:Epidermal Cyst / Q000150:complications / Q000473:pathology; D004817:Epidermis / Q000473:pathology; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D017495:Hyperpigmentation / Q000150:complications* / Q000235:genetics / Q000473:pathology; D017496:Hypopigmentation / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D012867:Skin / Q000473:pathology; D012868:Skin Abnormalities / Q000150:complications / Q000473:pathology; D012871:Skin Diseases / Q000150:complications / Q000473:pathology; D017444:Skin Diseases, Papulosquamous / Q000150:complications* / Q000235:genetics / Q000473:pathology