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 hereditary hemorrhagic telangiectasia.
- Porphyria cutanea tarda can have sclerodermatous face and hand lesions.
- Calcinosis cutis can occur in dermatomyositis (especially the juvenile form), and it may have prominent red and purple atrophic lesions that can be confused with SSc.
- Graft-versus-host disease should be clear from history of a transplant.
- Scleromyxedema often has a monoclonal gammopathy.
- CREST syndrome may also be confused with scleredema associated with diabetes mellitus or a streptococcal infection.
- Nephrogenic systemic fibrosis 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.
- Eosinophilic fasciitis presents with distal extremity sclerosis but lacks other features of SSc.