title: Dyschromatosis universalis hereditaria.
abstract: 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.
authors.affiliation: Department of Dermatology, PSG Institute of Medical Sciences & Research, Coimbatore, Tamil Nadu, India. kgsethu@yahoo.com
authors.forename: G
authors.initials: G
authors.lastname: Sethuraman
authors.forename: C R
authors.initials: CR
authors.lastname: Srinivas
authors.forename: M
authors.initials: M
authors.lastname: D'Souza
authors.forename: D Mohan
authors.initials: DM
authors.lastname: Thappa
authors.forename: L
authors.initials: L
authors.lastname: Smiles
mesh_terms: 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*