Limited cutaneous systemic sclerosis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

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.

Diffuse cutaneous systemic sclerosis (dcSSc): tight hand and fingers, increased pigmentation, thumb ulcer

From the personal collection of Professor J. Pope; used with permission

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Algorithm for diagnosis of systemic sclerosis
Characteristics of limited scleroderma versus diffuse scleroderma
Prayer sign: fingers cannot touch fully, note also digital tuft resorption
Dilated capillaries at the nailbeds
Calcinosis on second and third fingers in systemic sclerosis
Systemic sclerosis with severe digital ulcers
Systemic sclerosis with claw hand deformities (dorsal view)
Claw hands, palmar view; patient cannot extend fingers
Multiple telangiectasia, finger flexion deformity, pits on third finger, healed ulcers on second and fourth fingers
Diffuse cutaneous systemic sclerosis (dcSSc): tight hand and fingers, increased pigmentation, thumb ulcer
Sclerodactyly with increased and decreased pigmentation
Raynaud phenomenon in systemic sclerosis with well-demarcated white finger and blue fingers
Man's hand with systemic sclerosis: note loss of hair, sclerodactyly with skin involvement proximally
Multiple telangiectasia in systemic sclerosis
Eosinophilic fasciitis with peau d'orange

Citations

Key Articles

Mayes MD, Lacey JV Jr., Beebe-Dimmer J, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003 Aug;48(8):2246-55.[Abstract][Full Text]
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.[Abstract][Full Text]
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-39.[Abstract][Full Text]

Other Online Resources

Arthritis Foundation
International Scleroderma Network (ISN)
EUSTAR (EULAR Scleroderma Trials and Research)

Referenced Articles

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