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Segmental Neurofibromatosis
Robert M. Miller, MD;
Robert S. Sparkes, MD
Arch Dermatol. 1977;113(6):837-838.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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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
. . . [Full Text PDF of this Article]
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.
Footnotes
Reprint requests to Dermatology Service, Veterans Administration Wadsworth Hospital Center, Los Angeles, CA 90073 (Dr Miller).
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