A retired grocer, aged 54, is presented from the Massachusetts General Hospital. Brown spots have been present on the left side of his neck and on the right buttock since birth. At the ages of 2½, 9, 11, 14, and 22, he sustained fractures of the legs, mostly following trivial trauma. During the last 20 years he has lost 2 inches in height.
There are brown macules with a "coast of Maine" configuration on the left side of the neck and the right buttock. There is prominent protuberance of the occipital bone and the right upper orbital ridge, right scoliosis, and the right leg is one inch shorter than the left.
Complete blood count and urinalysis were normal. The Hinton reaction was negative.
Roentgenograms show cystic bony changes on the right side of the ribs, thickening of the bone at the base of the occiput, sphenoid, and right orbital bones.
What Is This (?). Arch Dermatol. 1961;83(2):325. doi:10.1001/archderm.1961.01580080155021
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