<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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.</p><p>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.</p></abstract>

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.

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

Medical Faculty, University of Niš, Serbia

Clinic of Skin and Venereal Diseases, Clinical Center of Niš, Serbia2

Serbian Journal of Dermatology and Venereology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

CREST sindrom - ograničena varijanta sistemske skleroderme: prikaz slučaja i pregled literature

Systemic scleroderma (SSc) is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and...

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

Publié en ligne: 06 janv. 2016

Pages: 97 - 114

DOI: https://doi.org/10.1515/sjdv-2015-0009

Mots clés
Scleroderma, Systemic, CREST Syndrome, Signs and Symptoms, Diagnosis

© 2015 Mirjana Paravina et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

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

Publié en ligne: 06 janv. 2016

Pages: 97 - 114

DOI: https://doi.org/10.1515/sjdv-2015-0009

Mots clésScleroderma, Systemic, CREST Syndrome, Signs and Symptoms, Diagnosis

© 2015 Mirjana Paravina et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Mots clés
Scleroderma, Systemic, CREST Syndrome, Signs and Symptoms, Diagnosis