What is Gorlin's syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirty-seven patients.
A series of 37 patients with Gorlin's syndrome is described; 23 had the typical naevi, 19 had jaw cysts and only 10 had both. A wide range of associated deformities was noted. The need for early identification of patients at risk is stressed; follow-up is essential for 2 reasons: The naevi although indistinguishable histologically from basal cell carcinomas only rarely require radical removal. The jaw cysts are locally invasive and should be radically removed when small. In 8 patients examined there was a low level of alpha-globulins in the plasma. Spontaneous involution of the naevi may occur in un-irradiated skin.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001845:Bone Cysts / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002280:Basal Cell Carcinoma / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007571:Jaw Diseases / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D008875:Middle Aged; D010375:Pedigree; D012272:Ribs / Q000002:abnormalities; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D013577:Syndrome