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August 1995

Segmental Neurofibromatosis: Is It Uncommon or Underdiagnosed?

Author Affiliations

Department of Dermatology Italian Navy Hospital "Giulio Venticinque" 74100 Taranto, Italy


Arch Dermatol. 1995;131(8):959-960. doi:10.1001/archderm.1995.01690200099023

Segmental neurofibromatosis (SNF) was described by Crowe et al1 in 1956 and termed sectorial neurofibromatosis. Some years later, Miller and Sparkes2 changed this term to SNF. The clinical peculiarities of SNF were established by Riccardi3 in his first classification: cafe au lait (CAL) spots and/or neurofibromas in a single unilateral segment of the body, with no crossing of the median line, no familial history, and no systemic involvement. Riccardi included SNF in his neurofibromatosis (NF) type V.

Over the years, the number of published cases of SNF have increased, but not all of the cases published were strictly in conformity with Riccardi's classification. Thus, Roth et al4 subdivided the SNFs into four subtypes: true segmental, localized with deep involvement, hereditary, and bilateral.

Report of a Case.  A 17-year-old man presented for the check up provided for enlisted men in the Italian Navy. From early childhood, he

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