Sethuraman G, Srinivas C R, D'Souza M, Thappa D Mohan, Smiles L
Department of Dermatology, PSG Institute of Medical Sciences & Research, Coimbatore, Tamil Nadu, India.
Clin Exp Dermatol. 2002 Sep;27(6):477-9. doi: 10.1046/j.1365-2230.2002.01129.x.
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.
遗传性泛发性色素异常症是一种临床异质性疾病。我们报告了两名患有遗传性泛发性色素异常症的印度非亲缘患者，他们的皮肤出现了全身性、进行性的网状色素沉着和色素减退。口腔黏膜和舌头也有斑驳的色素沉着。有趣的是，手掌和脚底也受到影响，表现为弥漫性色素沉着，其间散布着斑点状色素脱失斑。其中一例可见伴有翼状胬肉形成的营养不良性甲改变。组织病理学显示有不同程度的色素失禁。虽然这种疾病的确切病因尚不清楚，但临床病理表现提示黑素小体或黑色素加工存在内在异常。
