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Article
June 1977

Segmental Neurofibromatosis

Author Affiliations

From the Veterans Administration Wadsworth Hospital Center, Los Angeles (Dr Miller) and the Divisions of Dermatology (Dr Miller) and Medical Genetics, (Dr Sparkes), University of California at Los Angeles School of Medicine.

Arch Dermatol. 1977;113(6):837-838. doi:10.1001/archderm.1977.01640060133020
Abstract

Neurofibromatosis (von Recklinghausen disease) is an autosomaldominant disorder with a frequency of about 1 in 3,000.1 It is characterized by abnormal cutaneous pigmentation and by numerous tumors that develop in association with elements of both the CNS and peripheral nervous system. The pigmented spots are known as café au lait spots; the tumors are neurofibromas. Both manifestations tend to be multiple and widely distributed over the body; and because the manifestations affect the skin, these problems are initially brought to the attention of the dermatologist. However, there has been relatively little attention paid in the dermatological literature to segmental cases of the disorder. We are reporting two such instances to emphasize the limited clinical manifestations and their possible implications.

Report of Cases 

Case 1.—  A 15-year-old girl sought help for the treatment of acne. Examination results also disclosed multiple pigmented macules in her right axilla (Crowe sign). Several larger

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