Multiple cutaneous neurilemmomas as a skin manifestation of neurilemmomatosis

Four cases of neurilemmomatosis with multiple cutaneous neurilemmomas are described, including clinical, histologic, and electron microscopic features. Thirteen definite cases and sixteen probable cases of neurilemmomatosis with cutaneous tumors were collected from the Japanese literature. The skin lesions of multiple cutaneous neurilemmomas are nodules, infiltrated pigmented lesions, and plaque-like lesions consisting of papules. From the findings presented here, neurilemmomatosis is a clinical entity clearly distinguished from the neurofibromatosis of von Recklinghausen's disease.