Limited cutaneous systemic sclerosis

Limited cutaneous systemic sclerosis is a subset of systemic sclerosis characterized by skin fibrosis of the fingers (sclerodactyly) and, in some cases, of the face and neck or the skin distal to the elbows and/or knees. It does not affect the upper arms, upper legs, or trunk.

Previously known as CREST syndrome, which stands for the presence of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. This term is rarely used now as CREST features are not limited to this type of systemic sclerosis.

Presence of anticentromere antibody implies better prognosis and longer survival.

Treatment is targeted to symptoms, or to organs involved, and to influencing the fibrosis of the skin and other connective tissues.

Renal crisis will be encountered by a small number of patients only; ACE inhibitors reduce the likelihood of renal failure, dialysis, and death from this complication.

Systemic sclerosis (a type of scleroderma) is an autoimmune connective tissue disease characterized by the production of autoantibodies (e.g., antinuclear antibody, anticentromere antibody, and antitopoisomerase I antibody) and the overproduction of collagen, which causes fibrosis of the skin and organs as well as vasculopathy with Raynaud phenomenon and obliteration of blood vessels.

There are two main types of systemic sclerosis: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). The two types can be differentiated by the extent of skin involvement.[1]LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988 Feb;15(2):202-5. http://www.ncbi.nlm.nih.gov/pubmed/3361530?tool=bestpractice.com lcSSc is characterized by skin fibrosis of the fingers (sclerodactyly) and, in some cases, of the face and neck or the skin distal to the elbows and/or knees. It does not affect the upper arms, upper legs, or trunk. In contrast, dcSSc also affects the trunk and the skin distal and proximal to the elbows and/or knees. The presence of anticentromere antibodies is uncommon in patients with dcSSc; however, antitopoisomerase I and anti-RNA polymerase III antibodies are more common in dcSSc compared with lcSSc. Systemic sclerosis sine scleroderma is a third, less common type of systemic sclerosis where there is no skin involvement but other features of systemic sclerosis are present. This topic focuses on the evaluation and management of lcSSc.

CREST syndrome is defined by the presence of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.[2]Steen VD, Wigley F, Hummers LK, et al. Systemic sclerosis. In: Hochberg MC, Silman AJ, Smolen JS, et al., eds. Rheumatology volume two. 3rd ed. Edinburgh: Mosby; 2003:1455-513.[3]Barnett AJ, Miller M, Littlejohn GO. The diagnosis and classification of scleroderma (systemic sclerosis). Postgrad Med J. 1988 Feb;64(748):121-5. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2428791/pdf/postmedj00182-0026.pdf http://www.ncbi.nlm.nih.gov/pubmed/3050937?tool=bestpractice.com [4]Winterbauer RH. Multiple telangiectasia, Raynaud's phenomenon, sclerodactyly, and subcutaneous calcinosis: a syndrome mimicking hereditary hemorrhagic telangiectasia. Bull Johns Hopkins Hosp. 1964 Jun;114:361-83. http://www.ncbi.nlm.nih.gov/pubmed/14171636?tool=bestpractice.com [5]Rodnan GP, Jablonska S, Medsger TA. Classification and nomenclature of progressive systemic sclerosis. Clin Rheum Dis. 1979;5:5-13.[6]Maricq HR, Valter I. A working classification of scleroderma spectrum disorders: a proposal and the results of testing on a sample of patients. Clin Exp Rheumatol. 2004 Jan-Feb;22(3 suppl 33):S5-13. http://www.ncbi.nlm.nih.gov/pubmed/15344590?tool=bestpractice.com [7]Masi AT; Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum. 1980 May;23(5):581-90. http://onlinelibrary.wiley.com/doi/10.1002/art.1780230510/pdf http://www.ncbi.nlm.nih.gov/pubmed/7378088?tool=bestpractice.com Previously, the term was used to label patients with lcSSc; however, as CREST syndrome can occur in both lcSSc and dcSSc, the use of the term is now considered inaccurate, and it is not commonly used.