Denouement and Discussion
(Bourneville-Pringle Syndrome, Epiloia)
The major manifestations include seizures, adenoma sebaceum, and mental retardation. The skin lesions are pink to reddish-orange nodules which usually appear before the age of 10 and may be present in the first year of life. They are located mainly on the face, usually with a butterfly distribution on the cheeks although all areas of the face can be involved. Histologically, these lesions show vascular and connective tissue changes and little involvement of the sebaceous glands. A characteristic lesion, the shagreen patch, is usually located on the trunk and consists of a slightly elevated area variable in size with increased or decreased pigmentation and an "orange peel" appearance. Fibromas occur around the nails, forehead, scalp, and gums. Other abnormalities include graying of the hair, leukoderma, cafe au lait spots, hemangiomas, and bronzing of the skin. The majority of the patients are
Gellis SS, Feingold M. Picture of the Month. Am J Dis Child. 1968;115(1):57–58. doi:10.1001/archpedi.1968.02100010059010
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