Pigmented xerodermoid--report of three cases.
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.
Das
Jayanta Kumar
JK
Department of Dermatology, Vivekananda Institute of Medical Sciences and Ramakrishna Mission Seva Pratisthan Hospital and 99, Sarat Bose Road, Kolkata--700 026, India. jayanta_das@hotmail.com
Gangopadhyay
Asok Kumar
AK
D000287:Administration, Topical; D000293:Adolescent; D000328:Adult; D001707:Biopsy, Needle; D005260:Female; D005500:Follow-Up Studies; D006016:Glycolates / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D007194:India; D008297:Male; D012720:Severity of Illness Index; D012871:Skin Diseases / Q000175:diagnosis* / Q000628:therapy; D013473:Sunscreening Agents / Q000627:therapeutic use; D014466:Ultraviolet Rays / Q000009:adverse effects; D014983:Xeroderma Pigmentosum / Q000175:diagnosis* / Q000628:therapy
D006016:Glycolates; D013473:Sunscreening Agents; C031149:glycolic acid