IN CUTANEOUS medicine, von Recklinghausen's neurofibromatosis is recognized as a complex of macular pigmented areas and tumors, either cutaneous or subcutaneous, often accompanied by a number of polymorphous peripheral and central abnormalities. The initial descriptions of the physical and pathological findings in this disease, as given by Rayer,1 Smith,2 and von Recklinghausen,3 did not include the pigmentary changes. The addition of these changes to the primary complex may be credited to Marie and Bernard4 and to Chauffard.5 The macular pigmented areas associated with neurofibromatosis are melanic in origin, are located in and around the basal cell layer of the epidermis, and are commonly called café-au-lait spots, because of their characteristic color—that of coffee with milk. café-au-lait spots may vary in size and configuration, may be single or multiple, and may be located on any part of the body. However, in contrast to the ephelides, they are more prevalent in the
CROWE FW, SCHULL WJ. DIAGNOSTIC IMPORTANCE OF CAFÉ-AU-LAIT SPOT IN NEUROFIBROMATOSIS. AMA Arch Intern Med. 1953;91(6):758–766. doi:10.1001/archinte.1953.00240180067008
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