A case of segmental neurofibromatosis

Abstract

Neurofibromatosis(NF) is a heterogeneous disorder clinically characterized by the presence of neurofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules. It is usually expressed as a generalized form, but is rarely localized in a limited part of the body as a segmental form. In 1982, Riccardi classified NF into 8 types and in a segmental NF(type V) the cutaneous lesions are limited to a single body segment, often in a unilateral, dermatomal disposition. We report a 45-year-old Korean woman who presented with several grouped soft, fresh red colored papules and nodules on the left side of the neck(left C2, C3 dermatomes). There were no café-au-lait spots, axillary freckles, Lisch nodules or family history. A biopsy specimen of cutaneou nodules showed circumscribed non-encapsulated tumor of the dermis was composed of loosely spaced spindle cells with elongated, wavy nuclei and thin, wavy collagenous strands. On the basis of clinicopathologic examination we diagnosed as segmental NF.