Dyschromatosis Universalis Hereditaria (DUH) is a form of dyschromatosis, a group of cutaneous conditions characterized by the simultaneous presence of both hyper- and hypo-pigmented macules, many of which are small in size and irregular in shape. DUH is a clinically heterogeneous disorder typified by a generalized distribution of this mottled pigmentation, presenting as a generalized leukomelanoderma, with relative sparing of the face, palms, and soles. The age of onset of these lesions is usually in the first few months of life. Some affected patients have shown systemic abnormalities, including short stature, high frequency deafness, erythrocyte, platelet and tryptophan metabolism abnormalities, bilateral glaucoma, cataract, and grand mal seizures.
Histopathology of the lesions shows a variable degree of pigmentary incontinence. The exact etiology of this disorder is unknown. However, studies seem to point towards an inherent abnormality of melanosomes or a defect in melanocyte processing. DUH occurs most commonly among the Japanese. However, there have been sporadic reports from other populations as well.