CREST syndrome diagnosed by oral lesions: A case report and review of the literature.

BACKGROUND: Calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is an acronym for the clinical features that are seen. Its etiology is unknown, affecting women three times more than men. CREST syndrome is often diagnosed by systemic symptoms. However, oral manifestations could be helpful in the early diagnosis of the disease. Less than 20 cases of CREST syndrome with oral symptoms were described.

OBJECTIVE: To report a case of a 26-year-old female, who was diagnosed with CREST syndrome based on findings of the oral mucosa.

CASE REPORT: Clinical examination revealed sclerodactyly and nail alterations. Oral findings were associated with hypochromic and paleness mucosa. The tongue was strongly rigid and reddish areas compatible with telangiectasias were found. The mouth opening was particularly compromised. Histological findings were suggestive of systemic sclerosis in the context of CREST syndrome. Considering the available diagnosis criteria, this case was diagnosed as CREST syndrome based on oral manifestations.

CONCLUSIONS: Oral manifestations of CREST syndrome could help in early clinical diagnosis, preventing a delay in the onset of an aggressive form of the disease. Accurate referrals of patients to specialists are needed for a multidisciplinary approach.