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September 1969

Two Unusual Neurocutaneous Disorders With Facial Cutaneous Signs

Author Affiliations

Los Angeles
From the Division of Neurology, Childrens Hospital of Los Angeles, University of Southern California (Dr. Sugarman), and the Department of Dermatology, University of California School of Medicine (Dr. Reed), Los Angeles.

Arch Neurol. 1969;21(3):242-247. doi:10.1001/archneur.1969.00480150032003

CUTANEOUS lesions of the face frequently are signs of associated cerebral or neurological disease. Well-known examples are tuberous sclerosis, Klein-Waardenberg syndrome, Sturge-Weber-Demitri syndrome, and to a lesser extent, facial hemiatrophy (Romberg-Parry syndrome). We would like to report two cases in which cutaneous signs were associated with severe mental deficiency and epilepsy. One patient had a nevus sebaceous of the mixed type, and the other had a cutaneous segmental depigmentation of the face, not previously described in the literature.

Report of Cases  Case 1.—A 12-year-old Chinese girl was seen in 1967 at the Childrens Hospital Clinical Research Center. At that time, she had been institutionalized for ten years at Pacific State Hospital for profound mental retardation.

History.—  The patient was the third child of a 26-year-old Chinese woman who had had a normal pregnancy, labor, and delivery. The child weighed 2,978 gm (6 lb 9 ounces) at birth. Head circumference,