Lower Extremity Skin Ulcer Associated With Neurofibromatosis Type 1: A Case Report
Gracia-Cazaña, T.; de la Fuente, S.; Concellón, Mía.A.; Ara-Martín, M.
Wounds a Compendium of Clinical Research and Practice 31(10): E65-E67
2019
ISSN/ISBN: 1044-7946 PMID: 31730516 Document Number: 698579
The association between neurofibromatosis type 1 (NF-1) and vasculopathy has been reported frequently, especially cerebral, intestinal, and peripheral vasculopathy. However, cutaneous vasculopathy is infrequent. The authors present the case of a 32-year-old man with a painful ulcer on his left thigh of 3 weeks' duration in the same location as a long-time capillary malformation associated with alopecia. The skin biopsy showed signs of perivascular fibromuscular dysplasia with proliferating myofibroblastic cells. The patient had been treated with various therapeutic options, such as topical antibiotics, oral and intralesional corticosteroids, and oral cyclosporine and intravenous prostanoids. Cutaneous vasculopathy related to the skin, such as livedo reticularis and ulcers of torpid evolution due to cutaneous vasculopathy are extremely rare. Thus, it is necessary to include skin ulcers as one of the phenotypic manifestations of NF-1.