Neurofibroma
A neurofibroma is benign, slow-growing peripheral nerve tumor that occurs most frequently in neurofibromatosis type 1 (NF1, von Recklinghausen’s disease). Neurofibromas occur as solitary or multiple lesions and can arise from any nerve; they most commonly involve skin, major nerve plexuses and large nerve trunks (plexiform neuroma). Cutaneous neurofibromas are soft, usually flesh colored noddules and are usually multiple.(1,2) Pressure on a cutaneous neurofibroma can often cause it to disappear through what seems to be a defect in the skin (buttonhole sign). Other common findings in NFs include café-au-lait spots, freckling in the axilla or groin, and Lisch nodules in the iris. There is an increased risk of benign and malignant tumors because the mutated neurofibromin does not function effectively as an antioncogene. NF1 is transmitted as an autosomal dominant and these hands are those of a mother and her daughter.
References
1. Campbell WW. Clinical signs in neurology : a compendium. Philadelphia: Wolters Kluwer Health, 2016. 2. Williams, VC, Lucas, J, Babcock, MA, et al. Neurofibromatosis type 1 revisited. Pediatrics. 2009; 123:124-133.
