Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete
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Xeroderma pigmentosum comprises a heterogeneous group of autosomal recessive hereditary diseases, which are characterized by a number of clinical characteristics and an abnormal DNA repair mechanism. Patients affected show a high frequency of mucocutaneous malignant tumors, especially squamous cell carcinomas and basal cell carcinomas. We report on a 65-year-old patient who successively developed a total of 15 malignant melanomas, 1 squamous cell carcinoma and 1 lymph node metastasis of a malignant melanoma. The clinical diagnosis of xeroderma pigmentosum was confirmed by the complementation analysis, which defined our patient as xeroderma pigmentosum of the complementation group D.