Dermal hyperneury

Abstract

Dermal hyperneury (DN) is a rarely reported form of small nerve hypertrophy characterized by an exaggerated size and prominence of dermal nerve fibers. Clinically, it can present with or without visible lesions, within a syndrome or sporadically, and in solitary or multiple fashion. The syndromes most commonly associated with DN include multiple endocrine neoplasia 2B and 2A, neurofibromatosis type II, and Cowden syndrome. Patients with syndrome-associated DN have an increased risk for various malignancies. Sporadic DN has been reported either in association with cutaneous tumors/reactive lesions, such as nodular prurigo, or in idiopathic form, where it can present with multiple DN lesions. There is a morphologic overlap between mucocutaneous neuromas and DN, as the former can have the appearance of either bulkier circumscribed lesions or tortuous and hyperplastic nerves more akin to DN. Epithelial sheath neuroma also shares a similar appearance to DN, from which it can be distinguished by a squamoid-appearing and thickened perineurium.