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Sclerosing Segmental Neurofibromatosis
Author(s) -
Lee Joong Sun,
Kim You Chan
Publication year - 2005
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.2005.tb00768.x
Subject(s) - neurofibromatosis , neurofibroma , medicine , neurofibromatoses , café au lait spot , pathology , anatomy , dermatology
Segmental neurofibromatosis is a rare disorder characterized by café‐au‐lait macules and/or neurofibromas limited to a single body segment. The neurofibromas in segmental neurofibromatosis are usually soft, non‐tender nodules as in other types of neurofibromatosis. Histopathologically, they are usually non‐encapsulated, loosely textured dermal tumors. We report a case of sclerosing segmental neurofibromatosis, in which the patient presented with several grouped, erythematous to brownish, firm tender nodules on the left side of the posterior neck. Histopathologically, the stroma was mostly very fibrotic, especially around hair follicles, in addition to the usual features of neurofibroma. The atypical clinical feature, hardness, and tenderness of the lesions may be associated with the fibrosis.