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Figure 18a.  Progressive skeletal changes. (a, b) Anteroposterior radiographs of the left foot in a patient at ages 2 years (a) and 5 years (b) show progressive and disproportionate growth of the metatarsal and phalangeal bones of the toes, macrodactyly and clinodactyly, and a cerebriform connective-tissue nevus (arrowheads in b). (c, d) Posteroanterior radiographs of the left hand in another patient at ages 4 years (c) and 16 years (d) show asymmetric and irregular overgrowth of the phalanges, more marked and with accompanying ankylosis of the interphalangeal joints in d.