Dyschromatosis universalis hereditaria.

Published on Sep 1, 2002in Clinical and Experimental Dermatology1.771
· DOI :10.1046/j.1365-2230.2002.01129.x
G Sethuraman5
Estimated H-index: 5
Mariette D'Souza7
Estimated H-index: 7
+ 2 AuthorsL. Smiles2
Estimated H-index: 2
(PSG Institute of Medical Sciences and Research)
Summary Dyschromatosis universalis hereditaria is a clinically heterogenous disorder. We report two unrelated Indian patients with dyschromatosis universalis hereditaria, who had generalized and progressive reticulate hyper- and hypo-pigmentation of the skin. The oral mucosa and tongue also showed mottled pigmentation. Intriguingly, the palms and soles were also affected with a diffuse hyper-pigmentation interspersed with spotty de-pigmented macules. Dystrophic nail changes with pterygium formation were seen in one case. Histopathology revealed a variable degree of pigmentary incontinence. Although the precise aetiology of this disorder is not yet known, the clinicopathological findings implicate an inherent abnormality of melanosomes or melanin processing.
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