Spontaneous Compartment Syndrome of the Hand in Systemic Sclerosis

Compartment syndrome refers to a condition of compromised circulation within a limited space due to increased pressure within that space. The reduced tissue perfusion results in reduced venous drainage, leading to increased interstitial tissue pressure and subsequent compromised arterial flow. Although not as common as compartment syndrome of the leg and forearm, compartment syndrome of the hand is not rare and can lead to devastating sequelae as a result of tissue necrosis. Compartment syndrome of the hand has several etiologies, including trauma, arterial injury, thermal injury, and constrictive bandaging. The cardinal clinical sign is pain that is aggravated by passive stretching of the muscles within the involved compartments. Extremity function is usually restored with expeditious fasciotomy of the involved myofascial compartments, and complications, such as intrinsic muscular dysfunction and Volkmann's ischemic contracture, can usually be prevented. There are no reported cases of compartment syndrome of the hand in patients with systemic sclerosis or Raynaud's phenomenon. Systemic sclerosis is a form of scleroderma that affects the skin and internal organs. The limited cutaneous subset affects the skin of the extremities but is associated with a set of characteristic features that includes calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. This report describes an unusual case of a patient who had spontaneous compartment syndrome of the hand. The patient's concomitant limited cutaneous systemic sclerosis may have played a role in this unusual occurrence. The diagnosis was based on the clinical picture, and the symptoms resolved after surgical decompression.