Dyschromatosis universalis hereditaria (DUH) is an autosomal dominant inherited rare genodermatosis wherein patient presents with hypopigmented and hyperpigmented macules of varying sizes in a reticulate pattern. We report a rare case of Dyschromatosis universalis hereditaria in a 23 year old male patient with no affected family members suggesting the possibility of sporadic mutation. Patient born of non consanguineous marriage presented with both hypopigmented macules and hyperpigmented keratotic papules with progressive diffuse hyperpigmentation over the trunk and both the extremities. Other system examination was normal. Histopathological examination showed pigment incontinence with collagenisation of the dermis. A diagnosis of Dyschromatosis universalis hereditaria was made based on history, clinical morphology and histopathology.
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