Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study
Francesco Zulian,Balu H. Athreya,Ronald M. Laxer,A. M. Nelson,S. K. Feitosa de Oliveira,Marilynn Punaro,Ruben Cuttica,Gloria C. Higgins,L.W.A. van Suijlekom-Smit,Terry L. Moore,Carol B. Lindsley,Julia Garcia-Consuegra,M. O. Esteves Hilário,Loredana Lepore,Clovis A. Silva,Célia Machado,Stella Garay,Yosef Uziel,Giorgia Martini,Ivan Foeldvari,A. Peserico,P Woo,John I. Harper +22 more
Reads0
Chats0
TLDR
The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence the efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.Abstract:
Objective. Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres.
Methods. A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS.
Results. Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr.
Conclusion. This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.read more
Citations
More filters
Journal ArticleDOI
Update on morphea: Part I. Epidemiology, clinical presentation, and pathogenesis
Nicole Fett,Victoria P. Werth +1 more
TL;DR: Morphea, also known as localized scleroderma, is a rare fibrosing disorder of the skin and underlying tissues as mentioned in this paper and is differentiated from systemic sclerosis based on the absence of sclerodactyly, Raynaud phenomenon, and nailfold capillary changes.
Journal ArticleDOI
En coup de sabre morphea and Parry-Romberg syndrome: A retrospective review of 54 patients
TL;DR: En coup de sabre morphea and Parry-Romberg syndrome frequently coexist and are likely both variants of morphea, and the efficacy of antimalarial agents and methotrexate in the treatment of these diseases remains unclear.
Journal ArticleDOI
Distinct Autoimmune Syndromes in Morphea: A Review of 245 Adult and Pediatric Cases
Justin J. Leitenberger,Rachael Cayce,Robert W. Haley,Beverley Adams-Huet,Paul R. Bergstresser,Heidi Jacobe +5 more
TL;DR: High prevalences of concomitant and familial autoimmune disease, systemic manifestations, and antinuclear antibody positivity in the generalized and possibly mixed subtypes suggest that these are systemic autoimmune syndromes and not skin-only phenomena.
Journal ArticleDOI
Pediatric morphea (localized scleroderma): review of 136 patients.
TL;DR: An increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms is suggested, are suggested in a large pediatric cohort from one center, 1989 to 2006.
Journal ArticleDOI
Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children.
TL;DR: Localized scleroderma or morphoea is often considered to be a benign self‐limiting condition confined to the skin and subcutaneous tissue, but the course of the disease is unpredictable and severe functional and cosmetic disability may result.
References
More filters
Journal ArticleDOI
Clustering of non-major histocompatibility complex susceptibility candidate loci in human autoimmune diseases
Kevin G. Becker,Richard M. Simon,Joan E. Bailey-Wilson,Boris Freidlin,William E. Biddison,Henry F. McFarland,Jeffrey M. Trent +6 more
TL;DR: A majority (approximately 65%) of the human positive linkages map nonrandomly into 18 distinct clusters, which supports a hypothesis that, in some cases, clinically distinct autoimmune diseases may be controlled by a common set of susceptibility genes.
Journal ArticleDOI
Classification of morphea (localized scleroderma)
TL;DR: Morphea represents a wide variety of clinical entities that seen to be on the opposite end of the scleroderma spectrum from systemic sclerosis and is proposed to be classified into the following five groups: plaque, generalized, bullous, linear, and deep.
Journal ArticleDOI
Methotrexate and corticosteroid therapy for pediatric localized scleroderma
TL;DR: Treatment with MTX and corticosteroids appears to be effective in the treatment of LS and is generally well tolerated, and a placebo-controlled study is necessary to confirm the efficacy of MTX therapy in LS.
Journal Article
Localized scleroderma in adults and children. Clinical and laboratory investigations of 239 cases
Angelo V. Marzano,Silvano Menni,Aurora Parodi,Alessandro Borghi,Alessandra Fuligni,Paolo Fabbri,Ruggero Caputo +6 more
TL;DR: Children and adults developed LS with analogous clinical and immunological features, however, the prevalence of LS variants differed between adult and pediatric populations, leading to different extracutaneous complications.
Journal ArticleDOI
Linear scleroderma. Clinical spectrum, prognosis, and laboratory abnormalities.
TL;DR: The clinical features and natural history of linear scleroderma in 53 patients and the laboratory tests helpful in the management of this disease are described in this article, where no patient had Raynaud's phenomenon or signs of systemic connective tissue disease in a mean follow-up of 10 years.
Related Papers (5)
Localized scleroderma in childhood is not just a skin disease.
Francesco Zulian,Cristina Vallongo,Patricia Woo,Ricardo Russo,Nicolino Ruperto,John I. Harper,Graciela Espada,Fabrizia Corona,Mukamel M,Richard Vesely,Elzbieta Musiej-Nowakowska,Jeff Chaitow,Joan Ros,Maria Teresa Apaz,Valeria Gerloni,Henryka Mazur-Zielinska,Susan Nielsen,Susanne Ullman,Gerd Horneff,Carine Wouters,Giorgia Martini,Rolando Cimaz,Ronald M. Laxer,Balu H. Athreya +23 more