Ischemic skin ulcers with neurofibromatosis 1 successfully treated with stent implantation.

We describe a case of intractable skin ulcers of the right leg caused by vasculopathy due to neurofibromatosis type 1 (NF1) in a 28-year-old Japanese man. He had been diagnosed with NF-1 according to multiple cafe-au-lait spots and neurofibromas on his trunk and extremities at the age of 10 years. Skin ulcers with edematous erythema were present on the patient's right lower thigh and upper heel for more than a month. Initially we diagnosed traumatic ulcer and cellulitis and started systemic antibiotics, but there was no improvement. The ulcers expanded, and new ulcers and erythema appeared on his right inguinal and popliteal region. Histopathology of the erythema showed the presence of neuron-specific enolase and S-100-positive spindle-shaped cells surrounding vessels, nerves, and appendages in the dermis and subcutaneous tissue, which had led to thrombosis and vessel obstruction. After the diagnosis of vasculopathy associated with NF1, we administered prostaglandin E1 and anticoagulant, but improvement was minor. Magnetic resonance angiography disclosed narrowed right common iliac, peroneal, and posterior tibial arteries. Following stenting according to these results, the ulcers gradually improved. In cases of intractable skin ulcers with NF1, we should take angiography to look for arterial abnormalities caused by NF1 and assign appropriate therapeutic intervention.