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Italian Journal of Dermatology and Venereology 2021 August;156(4):446-54

DOI: 10.23736/S2784-8671.20.06632-8

Copyright © 2020 EDIZIONI MINERVA MEDICA

language: English

A retrospective study on clinical subtypes and management of morphea in 10 Italian Dermatological Units

Annalucia VIRDI 1 , Annalisa PATRIZI 1, Stefano CAMBIAGHI 2, Andrea DIOCIAIUTI 3, May EL HACHEM 3, Donatella SCHENA 4, Andrea BASSI 5, Domenico BONAMONTE 6, Valeria BRAZZELLI 7, Anna BELLONI FORTINA 8, Patrizia PEPE 9, Vito DI LERNIA 10, Iria NERI 1

1 Division of Dermatology, Department of Experimental, Diagnostic and Specialty Medicine, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy; 2 Unit of Pediatric Dermatology, Department of Pathophysiology and Transplantation, Maggiore Polyclinic Hospital, IRCCS Ca’ Granda Foundation, University of Milan, Milan, Italy; 3 Dermatology Unit, Bambino Gesù Children’s Hospital IRCCS, Rome, Italy; 4 Section of Dermatology and Venereology, Department of Medicine, University of Verona, Verona, Italy; 5 Unit of Pediatric Dermatology Service, Anna Meyer Pediatric Hospital, Florence, Italy; 6 Section of Dermatology, Department of Biomedical Science and Human Oncology, University of Bari, Bari, Italy; 7 Department of Clinical-Surgical, Diagnostic and Pediatric Science, Institute of Dermatology, IRCCS San Matteo Polyclinic Foundation, University of Pavia, Pavia, Italy; 8 Unit of Pediatric Dermatology, Department of Medicine, University of Padua, Padua, Italy; 9 Unit of Dermatology, Surgical, Medical and Dental Sciences, Department of Morphological Sciences Related to Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy; 10 Unit of Dermatology, Arcispedale S. Maria Nuova IRCCS, Reggio Emilia, Italy



BACKGROUND: There are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience.
METHODS: A multicenter retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units.
RESULTS: We collected the data of 69 children affected by: circumscribed morphea (39.1%); linear morphea of trunk and limbs (14.5%); en coupe de sabre morphea (ECDS) (14.5%); progressive facial hemiatrophy (8.7%); generalized form (18.8%); mixed morphea (4.4%). The mean age at onset was 6.86±3.21 years, mainly between 2 and 8 years, but is statistically significantly lower for ECDS (4.5±3.03). Localizations were: head/neck (30.4%), limbs (26.1%), trunk (14.5%), 2 or more sites (29%), most often the trunk plus limbs. Extracutaneous manifestations were observed in 26.1% patients. 10 patients presented a second autoimmune disorder. Treatments were topical in 26.1% cases and systemic (alone or associated with topical treatments) in 68.1%.
CONCLUSIONS: There was a lack of uniformity in the management of patients and an increasing awareness of dermatologists on the use of systemic therapies, in particular of methotrexate, which is no longer exclusive to rheumatologists. Methotrexate causes stabilization and improvement of the clinical signs, but topical creams are still considered adjuvant or maintenance therapies during/after the use of systemic drugs.


KEY WORDS: Scleroderma, localized; Dermatologists; Child

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