Diffuse neurofibroma - an uncommon cause of alopecia
A 42-year-old Caucasian male presented with an asymptomatic swelling in the frontal and\nleft temporal regions of the scalp. The swelling had been present since he was 17 years\nold, gradually enlarging since then. The first skin biopsy, at the age of 29, was\ninconclusive and the patient was lost to follow-up; however, due to the continued growth\nof the swelling and loss of overlying hair, he returned years later for reevaluation.\nHe had no relatives with history of neurofibromatosis (NF).\nPhysical examination revealed a multicentric tumor with ill-defined borders composed of\nthree confluent plaques with 9cm, 8cm and 6cm diameter located in the frontal and left\ntemporal regions of the scalp (). The\ntumor was soft on palpation and there was loss of hair on the overlying, hyperpigmented\nskin. There were no changes in the underlying bone nor was there any bruit. The\nremaining physical examination aspects were unremarkable and no other NF features were\nfound. Ophthalmologic examination excluded the presence of Lysch nodules. The patient\nshowed no cognitive impairment. At this time, the diagnostic hypotheses were lipoma,\nvenous malformation and low-grade malignancy.\nUltrasound showed an ill defined, diffusely hyperechoic mass with 0.9cm thickness.\nDoppler revealed multiple vascular structures, suggestive of hemangioma. Cranial\ncomputed tomography (CT) showed no intracranial extension (). A biopsy specimen of the tumor revealed spindle cells\nwith oval nuclei in a myxoid background. The lesion was located in the dermis and\nsubcutaneous tissue; skin appendages were scarce in the deeper dermis. No Meissner\nbodies were seen. ( and ) These findings were suggestive of a diffuse\nneurofibroma and the patient was submitted to surgical excision of the tumor with a\ntemporal rotation flap and a free skin graft. The tumor was completely excised and the\npostoperative period was uneventful. Histopathological examination confirmed a diffuse\nneurofibroma. No recurrence was recorded during the first 6 months of follow-up.