TY - JOUR
T1 - Schwannomatosis: dermatological relevance of an unusual form of neurofibromatosis type 2 (NF‐2)
AU - Giménez‐Arnau, Ana Ma
AU - Pou‐Serradell, Adolf
AU - Camarasa, José G.
PY - 1992/1/1
Y1 - 1992/1/1
N2 - 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
AB - 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
KW - Neurilemmomatosis
KW - Neurofibromatosis
KW - Schwannomatosis
UR - https://www.scopus.com/pages/publications/0026988851
U2 - 10.1111/j.1468-3083.1992.tb00644.x
DO - 10.1111/j.1468-3083.1992.tb00644.x
M3 - Article
SN - 0926-9959
VL - 1
SP - 269
EP - 279
JO - Journal of the European Academy of Dermatology and Venereology
JF - Journal of the European Academy of Dermatology and Venereology
IS - 4
ER -