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

Author:

Gilligan Gerardo1ORCID,Leonardi Nicolás2,Sambuelli Gabriela3,Panico René12

Affiliation:

1. Oral Medicine Department Facultad de Odontología Universidad Nacional de Córdoba Cordoba Argentina

2. Oral Medicine Department School of Dentistry Health Sciences Faculty Universidad Católica de Córdoba Córdoba Argentina

3. General Pathology Department Clínica Universitaria Reyna Fabiola Córdoba Argentina

Abstract

AbstractBackgroundCalcinosis, 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.ObjectiveTo report a case of a 26‐year‐old female, who was diagnosed with CREST syndrome based on findings of the oral mucosa.Case reportClinical 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.ConclusionsOral 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.

Publisher

Wiley

Reference25 articles.

1. Beltrán‐VeraAD Cuzco‐MacíasAC Cuzco‐MacíasLG Obregón‐OrtegaLG Coloma‐Gaibor ML.Esclerosis Sistémica Progresiva con Síndrome de CREST. Reporte de Caso. Published online.2023. doi:10.5281/ZENODO.7605530

2. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes

3. CREST Syndrome

4. CREST Syndrome in Systemic Sclerosis Patients – Is Dystrophic Calcinosis a Key Element to a Positive Diagnosis?

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