REVIEW  RHEUMATOLOGIC DERMATO(PATHO)LOGY: HOW THE MICROSCOPE CAN HELP THE CLINICIAN Free access

Giornale Italiano di Dermatologia e Venereologia 2018 April;153(2):208-15

DOI: 10.23736/S0392-0488.18.05922-9

Copyright © 2018 EDIZIONI MINERVA MEDICA

lingua: Inglese

Scleroderma with an update about clinico-pathological correlation

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

¹ Dermatologic Clinic, Department of Public Health, University of Cagliari, Cagliari, Italy; ² Unit of Dermatology, Department of Health Sciences, Magna Graecia University, Catanzaro, Italy; ³ Department of Molecular Medicine, “Sapienza” University, 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 - Scleroderma, localized - Pathology