The risk of malignant melanoma, internal malignancy and mortality in xeroderma pigmentosum patients.
Mortality and malignancy were studied in a series of 32 patients with xeroderma pigmentosum presenting during the period 1950-84. Twenty-nine of the patients were followed to the end of 1984 using the National Health Service Central Register, and hospital case records and correspondence with referring physicians; three patients had incomplete follow-up. Three of the patients developed malignant melanoma during follow-up, but none developed internal malignancy. Two males and one female died [standardized mortality ratio (SMR) = 971, P less than 0.05, for males; SMR = 1931, not significant, for females]. All three deaths were from non-malignant causes: severe neurological involvement with terminal infection. This high mortality from neurological complications in xeroderma pigmentosum patients contrasts with previously reported mortality, particularly from actinic-induced cutaneous tumours.
English
J S
JS
St John's Hospital for Diseases of the Skin, London, U.K.
Swerdlow
A J
AJ
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008545:Melanoma / Q000150:complications*; D009422:Nervous System Diseases / Q000150:complications; D012307:Risk Factors; D012878:Skin Neoplasms / Q000150:complications*; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000401:mortality