Pigmented xerodermoid--report of three cases.

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2005-01-06
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Abstract
Pigmented xerodermoid, a rare genodermatosis, presents with clinical features and pathology similar to xeroderma pigmentosum, but at a later age. DNA repair replication is normal, but there is total depression of DNA synthesis after exposure to UV radiation. Two siblings in their teens and a man in his thirties with features of pigmented xerodermoid, e.g. photophobia, freckle-like lesions, keratoses, dryness of skin, and hypo- and hyper-pigmentation, are described. Although classically the onset of pigmented xerodermoid is said to be delayed till third to fourth decade of life, it seems the disease may appear earlier in the tropics. Early diagnosis and management could be life-saving.
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Das JK, Gangopadhyay AK. Pigmented xerodermoid--report of three cases. Indian Journal of Dermatology, Venereology and Leprology. 2005 Jan-Feb; 71(1): 41-3