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The risk of malignant melanoma, internal malignancy and mortality in xeroderma pigmentosum patients
Author(s) -
ENGLISH J.S.C.,
SWERDLOW A.J.
Publication year - 1987
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1987.tb04925.x
Subject(s) - xeroderma pigmentosum , medicine , malignancy , melanoma , dermatology , oncology , cancer research , genetics , biology , dna repair , dna
SUMMARY 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 < 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.