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November 1991

Neurofibromatosis Mimicry

Author Affiliations

The Neurofibromatosis Institute 11 W Del Mar Blvd Pasadena, CA 91105

Arch Dermatol. 1991;127(11):1714-1715. doi:10.1001/archderm.1991.01680100114016

I chose the title of this editorial carefully, so as to allow for two sets of considerations: first, other disorders mimicking neurofibromatosis (NF), and, second, NF mimicking other disorders.

The NFs as a group are defined on the basis of patchy hyperpigmentation of the skin, ie, the presence of one or more neural-crest-derived tumors on, in or subjacent to the skin, or both.1-3The most common form of NF, namely, von Recklinghausen NF, or NF-1, is characterized by the consistent presence of both sets of features; although in young children only pigmentation abnormalities, ie, cafe-au-lait spots (CLSs), may be present. Conversely, in the adult with NF-1 fading of the CLS may take place and only large numbers of cutaneous tumors remain as the cutaneous manifestation of the disorder.

For NF-1, the situation usually is quite clear. Six or more CLSs at any age must raise the possibility of NF-1. Even if this is the only clinical finding in a child,

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