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A Journal on Dermatology and Sexually Transmitted Diseases

Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2018 Jan 24

DOI: 10.23736/S0392-0488.18.05922-9


language: English

Scleroderma with an update about clinico-pathological correlation

Franco RONGIOLETTI 1 , Caterina FERRELI 1, Laura ATZORI 1, Ugo BOTTONI 2, Giuseppe SODA 3

1 Clinica Dermatologica, Dipartimento di Scienze della Salute e Sanità Pubblica, Università di Cagliari, Cagliari, Italy; 2 Dipartimento di Scienze Della Salute Sezione Dermatologia, Università Magna Graecia di Catanzaro, Catanzaro, Italy; 3 Dipartimento di Medicina Molecolare "Sapienza" Università di Roma, Rome, Italy


Scleroderma is divided into a systemic form called systemic sclerosis and a localized form also called morphea. According to 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis, developed by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR), skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for a patient to be classified as having scleroderma. Histological examination is not included in the diagnostic criteria and is not routinely performed. Skin biopsy is recommended only in the case of diagnostic doubt with other scleroderma like disorders (scleromyxedema, scleredema, nephrogenic systemic fibrosis). Alternatively, skin biopsy is also often performed for research purposes. Indeed, the first step analysis of new cytokines or pathways that may contribute to the pathogenesis of the disease requires the evaluation of their expression or activation in the skin of scleroderma patients compared to healthy controls. The histological picture of the skin in bot localized and systemic scleroder shows initially microvascular alterations and chronic inflammation while in the more advanced stages skin fibrosis prevails. Localized scleroderma (LS) or morphea includes a number of subtypes which are classified more according to their clinical presentation rather than histopathological pictures. However, some histopathologic changes may be useful in differentiating each entity from the others and from other sclerodermoid disorders.

KEY WORDS: Scleroderma - Systemic sclerosis - Morphea - Histopathology

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Publication History

Article first published online: January 24, 2018
Manuscript accepted: January 22, 2018
Manuscript received: January 18, 2018

Cite this article as

Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G. Scleroderma with an update about clinico-pathological correlation. G Ital Dermatol Venereol 2018 Jan 24. DOI: 10.23736/S0392-0488.18.05922-9

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