Unusual presentation of hereditary leiomyomatosis mimicking neurofibromatosis

Hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome is a rare, autosomal dominant condition that predisposes individuals to cutaneous leiomyomas (CLMs), renal cell carcinomas (RCCs), and uterine leiomyomas. In HLRCC, CLMs develop during adolescence or late adulthood as smooth-surfaced, skin-colored or pink-to-brown papules or nodules usually ranging from 0.2 to 2.0 cm in diameter and 1 to 150 in number.1, 2 Typical involvement includes the trunk, neck, face, and extensor surfaces. Lesions are painful in up to 90% of patients.1, 2 Predisposition to papillary RCC in affected patients necessitates evaluation for malignancy. We present a case of HLRCC initially thought to represent neurofibromatosis type 1 (NF-1).