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December 1979

Resident's Page

Author Affiliations

Coordinator University of California School of Medicine, San Diego

Arch Dermatol. 1979;115(12):1442-1444. doi:10.1001/archderm.1979.04010120040017

PATHOLOGIC QUIZ CASE 1  Leslie A. Mark, MD, San DiegoSince birth, a 24-year-old woman has had linear whorled hypopigmentation of the skin of her trunk and extremities (Fig 1 and 2). She was also noted to have grouped baggy herniations of fat covered by a thin layer of hypopigmented and hyperpigmented skin on the right part of her thorax and the posterior part of her right thigh. Other congenital lesions consist of microcephaly, depressed nasal bridge, with hypoplasia of the right part of the clavicle, four hypoplastic ribs, pectus excavatum, and radialulnar synostosis. Severe bilateral hand defects include supernumerary digits, syndactylism of the third and fourth fingers, single palmar crease, a broad bifid left thumb, rudimentary fifth finger, and camptodactyly of the right forefinger. During the first decade of life, patchy alopecia of the scalp, scoliosis, and angiofibromatous nodules of the lip and ocular canthi developed. She is now