Limited cutaneous systemic sclerosis
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Synopsis

Limited cutaneous systemic sclerosis (lcSSc) is a subtype of systemic sclerosis (SSc). Previously, the term CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias) was used to refer to patients with lcSSc. However, given that CREST syndrome features can occur in both lcSSc and diffuse cutaneous systemic sclerosis (dcSSc; the more generalized form of SSc) and many patients with lcSSc do not display all 5 features of CREST syndrome, lcSSc is now the favored term.
Patients with lcSSc can be differentiated from those with dcSSc by the limited skin involvement to distal extremities. Patients with lcSSC may also complain of dysphagia, chronic heartburn, dyspnea, joint contractures, and diminished exercise capacity. Patients have a lower frequency of cardiac and renal involvement and a more prolonged survival when compared to patients with dcSSc. Additionally, the anticentromere antibody is strongly associated with lcSSc.
There is also a rare subtype of SSc that lacks skin sclerosis but has Raynaud phenomenon, nail fold capillary changes, internal organ involvement, and positive serologies called SSc sine scleroderma that has a similar trajectory to lcSSc.
Patients with lcSSc can be differentiated from those with dcSSc by the limited skin involvement to distal extremities. Patients with lcSSC may also complain of dysphagia, chronic heartburn, dyspnea, joint contractures, and diminished exercise capacity. Patients have a lower frequency of cardiac and renal involvement and a more prolonged survival when compared to patients with dcSSc. Additionally, the anticentromere antibody is strongly associated with lcSSc.
There is also a rare subtype of SSc that lacks skin sclerosis but has Raynaud phenomenon, nail fold capillary changes, internal organ involvement, and positive serologies called SSc sine scleroderma that has a similar trajectory to lcSSc.
Codes
ICD10CM:
M34.89 – Other systemic sclerosis
SNOMEDCT:
298285004 – Limited cutaneous systemic sclerosis
M34.89 – Other systemic sclerosis
SNOMEDCT:
298285004 – Limited cutaneous systemic sclerosis
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Last Reviewed:09/09/2025
Last Updated:09/23/2025
Last Updated:09/23/2025
Limited cutaneous systemic sclerosis