[Disseminated cutaneous neurilemmomatosis]

Neurilemmomatosis is a rare, non-inherited disease which has several features similar to type I neurofibromatosis.

A 30-year-old man had more than 300 cutaneomucosal tumours. Most were sessile and of firm consistency on normal coloured skin. Histology reported masses of fusiform dermal cells S100 protein (+), NSE (-), actin (-). The cells had a polygonal ultrastructure with fine ramifications. Neurilemmomatosis (schwannomatosis) was diagnosed. No neurological or systemic disorder could be detected. The most bothersome tumors were destroyed.

This is a typical anatomoclinical presentation of cutaneous neurilemmomatosis. This exceptional state raises the problem of differential diagnosis and nosological distinction from von Recklinhausen's disease. A characteristic feature in this case was the total absence of any malformation or associated tumour, notably neurological tumours. To our knowledge, this is the only documented case of disseminated neurilemmomatosis strictly limited to the skin.