Secretan’s Syndrome of the Hand: Literature Review and Surgical Case Report of a Rarely Documented Condition
Listed in
This article is not in any list yet, why not save it to one of your lists.Abstract
Background: Secretan’s syndrome is a rare and under-recognized condition characterized by chronic, indurated, non-pitting edema of the dorsal hand with thumb sparing. Fewer than a few dozen cases have been reported worldwide, mostly as isolated case reports, and its pathogenesis remains debated between traumatic, inflammatory, and factitious mechanisms. This article presents a surgically managed hyperplastic case and a review of the literature, emphasizing how principles of precision medicine can guide diagnosis and treatment. Materials and Methods: A 36-year-old healthcare worker developed progressive dorsal swelling of the left hand following minor trauma, with marked restriction of metacarpophalangeal flexion. Laboratory tests and radiographs were normal. MRI demonstrated peritendinous fibrosis encasing the extensor tendons. Psychiatric evaluation excluded factitious behavior. Given the functional limitation and MRI evidence of fibrosis, selective dorsal fasciotomies and extensor tendon tenolysis were performed. A systematic literature review was conducted to summarize epidemiology, clinical and imaging features, histopathology, and management options. Results: Histology revealed fibro-adipose tissue with chronic inflammatory changes and CD68+ histiocytic aggregates; microbiological cultures were negative. Postoperative rehabilitation enabled significant functional recovery. The literature review confirmed the scarcity of published cases and the absence of standardized guidelines. MRI consistently proved to be the most informative imaging tool, while surgical treatment was described only in hyperplastic, refractory forms. Conclusions: This case and review illustrate how a precision medicine approach can optimize management of rare disorders. Early MRI-based diagnosis, multidisciplinary assessment, and phenotype-driven surgical intervention allowed tailored treatment and favorable outcome. Personalized care that integrates clinical features, imaging findings, and patient-specific factors may improve results in hyperplastic Secretan’s syndrome despite the limited evidence base.