Calcinosis universalis has been used to describe extensive areas of cutaneous calcification without underlying disease. Using modern diagnostic techniques, these cases usually can be diagnosed as dermatomyositis, scleroderma, scleroderma-like syndromes including graft-versus-host disease, and scleroderma / dermatomyositis overlap syndromes.
Other manifestations associated with CREST include pulmonary hypertension, autoimmune hepatitis, biliary cirrhosis, and sicca syndrome. Some patients may have what is termed "overlap disease," in which CREST occurs in conjunction with another autoimmune condition. CREST is more common in women, especially during middle age and the childbearing years. Certain ethnic groups are also predisposed, including Choctaw Native Americans and persons of Japanese descent.
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 scleroderma.
- 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.