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

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.

Damage-induced reactive oxygen species regulate vimentin and dynamic collagen-based projections to mediate wound repair.

Tissue injury leads to early wound-associated reactive oxygen species (ROS) production that mediate tissue regeneration. To identify mechanisms that function downstream of redox signals that modulate regeneration, a vimentin reporter of mesenchymal cells was generated by driving GFP from the vimentin promoter in zebrafish. Early redox signaling mediated vimentin reporter activity at the wound margin. Moreover, both ROS and vimentin were necessary for collagen production and reorganization into projections at the leading edge of the wound. Second harmonic generation time-lapse imaging revealed that the collagen projections were associated with dynamic epithelial extensions at the wound edge during wound repair. Perturbing collagen organization by burn wound disrupted epithelial projections and subsequent wound healing. Taken together our findings suggest that ROS and vimentin integrate early wound signals to orchestrate the formation of collagen-based projections that guide regenerative growth during efficient wound repair.