Gorlin's syndrome

(Robert James Gorlin, born 1923, American physician), or naevoid basal cell carcinoma syndrome, an autosomal dominant syndrome manifested by multiple basal cell carcinomas, brachydactyly, palmar pits, neurologic abnormalities, eye changes, dentigerous cysts, rib deformities (splaying, synostosis, bifid ribs) and spine abnormalities (kyphoscoliosis, spina bifida, spondylolysis, hemivertebrae). The metacarpal bones may also be shortened. Other findings include calcifications (falx cerebri, tentorium, dura mater), hypertelorism and partial agenesis of the corpus callosum. Small radiolucent flame-shaped cystic areas have been reported in the phalanges, humerus, radius and ulna.

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