FAMILIAL SYNDROMES WITH SKIN TUMOR-MARKERS

A number of interesting syndromes have been described in which skin tu mors are markers of heritable disorders. In Cowden's disease, Muir-Tor re's syndrome, and Gardner's syndrome, benign skin tumors accompany an d sometimes precede the development of internal visceral malignancy. T he association of skin cancers with other abnormalities is found in ne void basal cell carcinoma syndrome, Bazex syndrome, Rombo syndrome, xe roderma pigmentosum, dysplastic nevus syndrome, and epidermodysplasia verruciformis. Other genetic syndromes in which benign skin tumors her ald the existence of systemic diseases include neurofibromatosis, tube rous sclerosis, Haber's syndrome, and Buschke-Ollendorff syndrome. Dia gnosis of one of these syndromes may be ascertained by taking a thorou gh family history. Recognition of the skin tumor may trigger the prope r questions regarding family medical history. Diagnosis hinges upon th e physician having a high enough index of suspicion to link the appear ance of the skin lesions to the diverse manifestations accompanying th ese disorders. Recognition will also set the stage for appropriate gen etic counseling.