[Intracranial arteriovenous malformation associated with multiple cutaneous follicular hamartoma. A new syndrome]

A case of 32-year-old man with neurocutaneous syndrome has been reported. The clinical picture is characterized by dominance of lesions of central nervous system beginning at early lifeperiod of patients with intermittent slow progression and with a variety of different symptoms, and with presence of multifocal intracranial space-occupying process resulting death. The pathomorphological alterations consist of multiple congenital developmental anomalies including vascular hamartosis of central nervous system and hair follicular hamartosis of skin. It is suggested that the distinct syndrome seems to be a new one regarding both association of intracranial arteriovenous malformation with multiple cutaneous follicular hamartoma and the speciality of cutaneous damage itself, which differs from other hair follicular hamartomas hitherto described. The alteration of skin may represent a follicular hamartoma of generalized type with differentiation in Headington's classification. The literature is briefly reviewed.