Pathological Case of the Month | Congenital Defects | JAMA Pediatrics | JAMA Network
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Special Feature
May 2000

Pathological Case of the Month

Author Affiliations

From the Department of Dermatology, University of Utah Health Sciences Center, Salt Lake City



Arch Pediatr Adolesc Med. 2000;154(5):527-528. doi:10.1001/archpedi.154.5.527

A 4-YEAR-OLD white girl had an enlarged frontal bone at birth. She had reconstructive surgery, and findings from bone biopsy showed fibrous dysplasia. Physicians in the dermatology department were consulted because of an 18-month history of "growths" on both feet. On examination, she had a dysmorphic facies with frontal bossing and a broad nasal root. An epidermal nevus followed Blaschko lines on the right posterior neck (Figure 1). There was cerebriform hyperplasia of both soles, with macrodactyly most notable on the right second toe (Figure 2). A faint port-wine stain was also observed on the left upper lateral thigh. A specimen from shave biopsy was obtained from the hypertrophic soft tissue on the toe. There was marked, dense, dermal fibrosis with mild disorganization and minimal cellularity (Figure 3). The fibrosis was well visualized by trichrome stain (Figure 4).