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Chronic Exertional Compartment Syndrome Requiring Bilateral Fasciotomy: An Atypical Complication of Familial Stiff Skin Syndrome in a Father and Son.

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ABSTRACT Stiff skin syndrome (SSS) is a rare cutaneous disorder characterized by cutaneous fibrosis resulting in the early onset of thickened and indurated skin, joint mobility restrictions, and contractures. We… Click to show full abstract

ABSTRACT Stiff skin syndrome (SSS) is a rare cutaneous disorder characterized by cutaneous fibrosis resulting in the early onset of thickened and indurated skin, joint mobility restrictions, and contractures. We describe a father and son with familial SSS who presented with bilateral exertional pain and a confirmed diagnosis of chronic exertional compartment syndrome on 4-compartment pressure testing. Patients experienced restored functionality with bilateral 4-compartment fasciotomy. Chronic exertional compartment syndrome should be considered in the differential diagnosis of patients with SSS and chronic pain of the lower limbs.

Keywords: skin; syndrome; compartment; chronic exertional; compartment syndrome; exertional compartment

Journal Title: Annals of plastic surgery
Year Published: 2023

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