J Pers Med. 2025 Dec 1;15(12):586. doi: 10.3390/jpm15120586.
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. Twelve studies reporting 17 patients have been published 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 literature review, highlighting how precision medicine principles 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. Due to functional limitation and MRI evidence of fibrosis, selective fasciotomies and tenolysis were performed. A systematic literature review was conducted, in accordance with the PRISMA 2020 guidelines, 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 proved the most informative imaging tool, while surgery was described only in 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 despite the limited evidence base.
PMID:41440949 | PMC:PMC12733787 | DOI:10.3390/jpm15120586