Patients with lcSSc can be differentiated from those with dcSSc by the limited skin involvement to distal extremities. The anticentromere antibody, while only present in approximately 40% of individuals with lcSSc, is a laboratory test used to differentiate the two entities.
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.
M34.1 – CR(E)ST syndrome
31848007 – CREST syndrome
Differential Diagnosis & Pitfalls
- Telangiectasias on face and lips resemble .
- can have sclerodermatous face and hand lesions.
- can occur in (especially the ), and it may have prominent red and purple atrophic lesions that can be confused with SSc.
- should be clear from history of a transplant.
- often has a monoclonal gammopathy.
- CREST syndrome may also be confused with associated with diabetes mellitus or a streptococcal infection.
- results in diffuse fibrosis but lacks the other features of SSc. This most frequently occurs in individuals with renal disease who were given gadolinium contrast.
- presents with distal extremity sclerosis but lacks other features of SSc.