lcSSc

Limited cutaneous systemic sclerosis (lcSSc) is a subset of systemic sclerosis (scleroderma) in which skin thickening is confined to the face, hands, and distal arms and legs, with trunk involvement generally absent. Patients often have Raynaud’s phenomenon for years before skin changes. Organ involvement tends to appear later but can include the lungs, heart, esophagus, and kidneys. lcSSc is contrasted with the diffuse form, which has more extensive skin involvement and earlier organ disease.

Classic clinical features include sclerodactyly, digital ulcers, telangiectasias, and calcinosis, along with esophageal dysmotility and reflux. Pulmonary arterial hypertension (PAH) is a key complication in lcSSc and can impact survival; interstitial lung disease is less common than in the diffuse form but can occur. Gastrointestinal involvement, including reflux and dysmotility, is common and may contribute to nutritional issues.

Serology typically shows anticentromere antibodies, especially in the CREST-associated phenotype (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia). Anti-Scl-70 and RNA polymerase III antibodies are more frequent in diffuse disease. Nailfold capillaroscopy may reveal characteristic microvascular changes.

Diagnosis combines clinical assessment with skin scoring (modified Rodnan skin score) and supportive investigations. Lung evaluation uses high-resolution CT and pulmonary function tests; cardiac screening includes echocardiography and, if indicated, right-heart catheterization; renal involvement is monitored as part of routine follow-up.

Management is organ-directed and supportive. Treatments address Raynaud’s phenomenon and digital ulcers (vasodilators), PAH (endothelin receptor antagonists, PDE5 inhibitors, prostacyclins), and reflux or esophageal dysmotility (acid suppression, prokinetics). Immunosuppressants have limited evidence in lcSSc and are used selectively. Regular multidisciplinary follow-up is recommended to monitor progression and complications.