Oral and Maxillofacial Manifestations of Stiff Skin Syndrome: A Systematic Review

BACKGROUND: Stiff skin syndrome (SSS) is a rare disorder that presents with progressive, deep induration of the skin that can ultimately limit joint mobility. It typically presents without visceral or immunologic abnormalities. OBJECTIVE: This review investigates oral and maxillofacial manifestations in patients with stiff skin syndrome. STUDY DESIGN: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and was registered on PROSPERO (CRD420251269876). Studies published between 1970 and 2025 were reviewed. Eligible studies included original articles, cohort studies, case series, and case reports involving human subjects with stiff skin syndrome and presenting oral or maxillofacial features. RESULTS: Twenty-seven articles were included in our study, and 204 patients were affected by this disease overall. The age of onset of this syndrome was predominantly early childhood. The segmental form was predominant compared with the widespread form (73.5% versus 26.5%). Major sign was the indurated skin in all patients (100%). Hypertrichosis, hyperpigmentation, subcutaneous signs, and limited temporomandibular joint mobility were considered minor signs, found at respective rates of 10%, 5%, 5%, and 1%. CONCLUSION: Stiff skin syndrome should be considered systematically in any patient presenting with facial skin induration and should be investigated thoroughly, excluding any visceral or biological abnormalities.