CREST Syndrome - a Limited Form of Systemic Scleroderma: a Case Report and Literature Review

Systemic scleroderma (SSc) is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and fibrosis of the skin and internal organs. According to the simplest, though incomplete classification, there are two forms of SSc: diffuse and...

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Published inSerbian Journal of Dermatology and Venerology Vol. 7; no. 3; pp. 97 - 114
Main Authors Paravina, Mirjana, Stanojević, Milenko, Spalević, Ljiljana, Ljubisavljević, Dragana, Zlatanović, Zorana, Popović, Danijela
Format Journal Article
LanguageEnglish
Published De Gruyter Open 01.09.2015
Subjects
CREST sindrom
CREST Syndrome
Diagnosis
Dijagnoza
Pregled literature
Prikazi slučajeva
Scleroderma, Systemic
Signs and Symptoms
Sistemska skleroderma
Znaci i simptomi
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Summary:Systemic scleroderma (SSc) is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and fibrosis of the skin and internal organs. According to the simplest, though incomplete classification, there are two forms of SSc: diffuse and limited (formerly acrosclerosis). CREST syndrome is a subtype of limited SSc, characterized by: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. We present a patient with all the features of the CREST syndrome, which appeared at the age of 43 and lasted for 23 years. The patient presented with a gradual development of symptoms during the first ten years, from Raynaud’s phenomenon, skin sclerosis, calcinosis, telangiectasia, and esophageal dysmotility. The diagnosis was based on clinical findings and relevant diagnostic procedures. The article presents a literature review on the epidemiology, etiology, pathophysiology, clinical manifestations, various attempts at classification, diagnostic criteria, and therapeutic modalities. When classifying systemic scleroderma into two main types — diffuse and limited, with CREST syndrome as a variant of the latter, it should be pointed out that both types represent clinical forms of systemic sclerosis, share similar visceral involvement, laboratory abnormalities and course which is variable, as was the case in our patient.
ISSN:1821-0902
2406-0631
DOI:10.1515/sjdv-2015-0009