Schwannomatosis: dermatological relevance of an unusual form of neurofibromatosis type 2 (NF‐2)

Four patients (three women and one man) suffering from multiple subcutaneous, painful, nodular tumors, diagnosed as multiple schwannomas of peripheral nerves (MCN), are presented. Two had radicular and VIII cranial nerve schwannomas associated with meningioma. The other two patients had only MCN. These cases were obtained from a prospective study of 30 patients with clinical criteria of neurofibromatosis (NF). The main inclusion criteria were pathological nuclear magnetic resonance (3). In 19 cases histological studies of internal neurological tumors were carried out. The four cases reported here presented fewer than six ‘café au lait’ spots. No hereditary background was demonstrated in these patients. Clinical and genetic investigations were insufficient to affirm that MCN or schwannomatosis is a new entity or a new form of NF. Sporadic cases of NF‐2 appear more often than has been admitted before and these non‐inherited forms appear to be in cases associated with MCN. Copyright © 1992, Wiley Blackwell. All rights reserved