Localized severe scleroderma: A retrospective study of 26 pediatric patients

Matilde Beltramelli, Paolo Vercellesi, Adina Frasin, Carlo Gelmetti, Fabrizia Corona

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Juvenile localized scleroderma includes different conditions characterized by skin hardening with increased collagen deposition. Although juvenile localized scleroderma is considered a relatively benign disease, lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Furthermore, extracutaneous manifestations are described. We retrospectively analyzed a cohort of 26 patients with severe Juvenile localized scleroderma with particular attention to clinical features, therapy, and long-term outcome. A subgroup of three patients has been further evaluated with infrared thermography. Our findings were consistent with the current literature for demographic, laboratory, and clinical characteristics at disease onset, but, with our patients, the prevalence of extracutaneous manifestations was higher, thus confirming the potential for severe juvenile localized scleroderma to affect organs other than the skin, without increased risk of development toward systemic sclerosis. Correlation between various treatments and clinical endpoint showed that systemic therapy lead to a better outcome: in particular, methotrexate appeared the most effective drug, capable in halting the progression of the disease and sometimes inducing its regression.

Original languageEnglish
Pages (from-to)476-480
Number of pages5
JournalPediatric Dermatology
Volume27
Issue number5
DOIs
Publication statusPublished - Sep 2010

Fingerprint

Localized Scleroderma
Retrospective Studies
Pediatrics
Skin
Systemic Scleroderma
Subcutaneous Tissue
Dermis
Methotrexate
Cosmetics
Disease Progression
Collagen
Therapeutics
Demography
Bone and Bones
Muscles
Juvenile-onset scleroderma
Pharmaceutical Preparations

ASJC Scopus subject areas

  • Dermatology
  • Pediatrics, Perinatology, and Child Health

Cite this

Localized severe scleroderma : A retrospective study of 26 pediatric patients. / Beltramelli, Matilde; Vercellesi, Paolo; Frasin, Adina; Gelmetti, Carlo; Corona, Fabrizia.

In: Pediatric Dermatology, Vol. 27, No. 5, 09.2010, p. 476-480.

Research output: Contribution to journalArticle

Beltramelli, Matilde ; Vercellesi, Paolo ; Frasin, Adina ; Gelmetti, Carlo ; Corona, Fabrizia. / Localized severe scleroderma : A retrospective study of 26 pediatric patients. In: Pediatric Dermatology. 2010 ; Vol. 27, No. 5. pp. 476-480.
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