CREST syndrome - Nail and Distal Digit
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
- 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.