Dyschromatosis universalis hereditaria.
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.
Sethuraman
G
G
Department of Dermatology, PSG Institute of Medical Sciences & Research, Coimbatore, Tamil Nadu, India. kgsethu@yahoo.com
Srinivas
C R
CR
D'Souza
M
M
Thappa
D Mohan
DM
Smiles
L
L
D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000235:genetics / Q000473:pathology; D010859:Pigmentation Disorders / Q000235:genetics / Q000473:pathology*; D012873:Skin Diseases, Genetic / Q000473:pathology*