Small-Fiber Polyneuropathy: An Institutional Experience

Abstract

Small-fiber polyneuropathy (SFN) is an underdiagnosed disease characterized pathologically by the destruction of neurite fibers. Given the manifestations of this disease occur primarily in the skin, dermatologists play an important role in the identification and initial management of SFN. This work aims to aid this identification by detailed evaluation of the similarities and differences of it to other common diagnoses that can mimic it and are more commonly seen in daily practice. In our first research, we performed a retrospective evaluation of cutaneous manifestations among 301 patients with a biopsy-confirmed diagnosis of SFN. We found particular skin features, mostly located in body extremities. Of these, a “burning”- type of pain in distal limbs, as well as numbness, edema, skin color changes, and loss of distal hair are the cutaneous features most characteristic to SFN patients included. In our second study, we investigated if the addition of medications aimed at suppressing inflammation would confer additional benefit to the disease at a later stage when there is considerable neurite fiber decline. We found the addition of an immunomodulator appears to improve symptomatic outcomes even in SFN patients with sensory symptomology of small fiber dysfunction, whose etiology for SFN was mostly idiopathic. We provided our institutional experience with the management of this condition, as a starting point for future multi-center evaluations of SFN cases.