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Axillary Freckling as a Diagnostic Aid in Neurofibromatosis

Ann Intern Med. 1964;61(6):1142-1143. doi:10.7326/0003-4819-61-6-1142
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Although Smith's (1) original description of the disease in 1849 did not mention pigmentation, nor did von Recklinghausen (2) mention it in 1882, increased pigmentation in the form of cafe-au-lait spots has long been associated with multiple neurofibromatosis. The addition of the pigmentary changes to the primary complex of tumors may be credited to Marie and Bernard (3) and to Chauffard (4) in 1896. This pigment (5, 6), situated in and around the basal cell layer of the epidermis, is melanin. The melanocytes in these sites are more abundant than usual. At one time much importance was ascribed to the


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