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Juvenile amyopathic dermatomyositis

Claudia L Montoya, Martha L Gonzalez, Fabio E Ospina, Gabriel J Tobón
Coexistence between two or more immunological-mediated diseases implies a clinical and therapeutic challenge that is uncommon in the clinical field. We report a rare case of a young patient with amyopathic juvenile dermatomyositis and erythrodermic psoriasis, with an excellent response to ustekinumab.
January 18, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Edward J Oberle, Michelle L Bayer, Yvonne E Chiu, Dominic O Co
BACKGROUND: Pediatric patients can present with skin manifestations of dermatomyositis without overt weakness (clinically amyopathic juvenile dermatomyositis [JDM]), but it is unclear how often this happens and how often they have subclinical muscle inflammation. OBJECTIVE: Our goal was to determine the frequency of clinically amyopathic JDM and the frequency with which a thorough evaluation uncovers subclinical myositis at a single institution. METHODS: A retrospective review was performed of 46 patients diagnosed with JDM at Children's Hospital of Wisconsin...
November 4, 2016: Pediatric Dermatology
Manabu Fujimoto, Rei Watanabe, Yosuke Ishitsuka, Naoko Okiyama
PURPOSE OF REVIEW: In dermatomyositis, disease-specific autoantibodies now cover more than 70% of patients. These autoantibodies closely correlate with distinct clinical manifestations. In the past few years, extensive evidence has been accumulated on clinical significance of dermatomyositis-specific autoantibodies including autoantibodies against melanoma differentiation antigen 5 (MDA5), transcriptional intermediary factor 1 (TIF1), nuclear matrix protein 2 (NXP2), and small ubiquitin-like modifier activating enzyme (SAE)...
November 2016: Current Opinion in Rheumatology
Eun Jin Doh, Jungyoon Moon, Sue Shin, Soo Hyun Seo, Hyun Sun Park, Hyun-Sun Yoon, Soyun Cho
Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin lesions which were erythematous scaly patches on the knuckles and elbows, since her childhood. When skin biopsy was performed from a left knee nodule, liquid chalky discharge was observed...
June 2016: Annals of Dermatology
Lúcia Maria Arruda Campos, Adriana M E Sallum, Cintia Z Camargo, Luís Eduardo C Andrade, Cristiane Kayser
No abstract text is available yet for this article.
April 18, 2016: Revista Brasileira de Reumatologia
Ilaria Pagnini, Antonio Vitale, Carlo Selmi, Rolando Cimaz, Luca Cantarini
Juvenile inflammatory myopathies represent a heterogeneous group of rare and potentially fatal disorders of unknown aetiology, characterised by inflammation and proximal and symmetric muscle weakness. Beyond many similarities, specific clinical, laboratoristic and histopathologic features underlie different subsets with distinguishing demographic, prognostic and therapeutic peculiarities. Over time, several forms of inflammatory idiopathic myopathies have been described, including macrophagic myofascitis, immune-mediated necrozing myopathy and the spectrum of amyopathic dermatomyositis that include hypomyopathic dermatomyositis, inclusion body myositis and cancer-associated myositis occurring almost exclusively in adults...
February 2017: Clinical Reviews in Allergy & Immunology
Norimoto Kobayashi, Shunichiro Takezaki, Ichiro Kobayashi, Naomi Iwata, Masaaki Mori, Kazushige Nagai, Naoko Nakano, Mari Miyoshi, Noriko Kinjo, Takuji Murata, Kenji Masunaga, Hiroaki Umebayashi, Tomoyuki Imagawa, Kazunaga Agematsu, Shinji Sato, Masataka Kuwana, Masafumi Yamada, Shuji Takei, Shumpei Yokota, Kenichi Koike, Tadashi Ariga
OBJECTIVE: Rapidly progressive interstitial lung disease (RP-ILD) is a rare but potentially fatal complication of JDM. The aim of this study was to establish markers for the prediction and early diagnosis of RP-ILD associated with JDM. METHODS: The clinical records of 54 patients with JDM were retrospectively reviewed: 10 had RP-ILD (7 died, 3 survived), 19 had chronic ILD and 24 were without ILD. Routine tests included a high-resolution CT (HRCT) scan of the chest and measurement of serum levels of creatine phosphokinase, ferritin and Krebs von den Lungen-6 (KL-6)...
May 2015: Rheumatology
Luciena Cegatto Martins Ortigosa, Vitor Manuel Silva dos Reis
BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases...
September 2014: Anais Brasileiros de Dermatologia
M Auriemma, A Capo, G Meogrossi, P Amerio
Idiopathic immune myopathies (IIM) are an heterogeneous group of autoimmune muscle disorders characterized by progressive muscle involvement. Dermatomyositis (DM) is the most common form of IIM. It is a multisystem disorder characterized by symmetric proximal, extensor, inflammatory myopathy, vascular involvement and a characteristic cutaneous eruption. Six types of DM have been identified: idiopathic, juvenile (JDM), cancer-related other autoimmune diseases-related, iatrogenic DM and amyopathic DM. Cutaneous manifestations of DM are the most important aspect of this disease and can precede from several months to years muscle or systemic involvement...
October 2014: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Dimitri Poddighe, Lorenzo Cavagna, Valeria Brazzelli, Paola Bruni, Gian Luigi Marseglia
Juvenile dermatomyositis is an immune-mediated inflammatory multi-system disease involving mainly striated muscles and skin. Typical dermatological features are fundamental to establish the diagnosis, especially whenever the myopathy is very mild or absent, as it occurs in the form called as amyopathic juvenile dermatomyositis. Sometimes, systemic rheumatic diseases can develop a hyperferritinemia syndrome characterized by hemophagocytosis, namely macrophage activation syndrome, which represents a severe and life-threatening complication...
November 2014: Autoimmunity Reviews
A J van der Kooi, M de Visser
Idiopathic inflammatory myopathies (IIMs), except for sporadic inclusion body myositis (sIBM), present with subacute symmetrical weakness of the limb girdle muscles, an elevated serum creatine kinase activity, and inflammatory cells in the muscle biopsy (necrotizing autoimmune myopathy being an exception). In dermatomyositis, additional skin abnormalities are found. IIMs are nowadays subclassified into the following categories: (1) dermatomyositis (DM), including (1a) classic dermatomyositis, which may be associated with connective tissue disorders (CTDs) and malignancy, (1b) juvenile dermatomyositis, and (1c) clinical amyopathic dermatomyositis; (2) polymyositis (PM) encompassing (2a) classical PM and (2b) nonspecific or overlap myositis, associated with CTD; (3) autoimmune necrotizing myopathy, associated with malignancy, statin use and CTD; and (4) sporadic IBM, sometimes associated with CTDs...
2014: Handbook of Clinical Neurology
V Shahi, D A Wetter, B M Howe, M D Ringler, M D P Davis
BACKGROUND: Studies on the radiological findings of calcinosis cutis in patients with autoimmune connective tissue disease (ACTD) are limited. OBJECTIVES: To describe imaging findings and radiological patterns of calcinosis cutis occurring with ACTD. METHODS: We retrospectively reviewed the cases of 37 patients with imaging studies assessing for calcinosis cutis associated with ACTD at our institution between 1996 and 2009. A formal blinded review of available images (i...
May 2014: British Journal of Dermatology
Sarah Tansley, Harsha Gunawardena
The idiopathic inflammatory myopathies: polymyositis (PM) and dermatomyositis (DM) have been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease and internal organ involvement. Over the last few years, it has become increasingly apparent that using a clinico-serological approach, both DM and PM can be defined into more homogeneous subsets. A large number of antibodies are directed against cytoplasmic or nuclear components involved in key regulatory intra-cellular processes including protein synthesis, translocation and gene transcription within this disease spectrum...
December 2014: Clinical Reviews in Allergy & Immunology
Manabu Fujimoto
Idiopathic inflammatory myopathies are a group of acquired skeletal muscle diseases that include polymyositis, dermatomyositis, and inclusion body myositis. Studies have shown many myositis-specific autoantibodies (MSAs) that are useful for the diagnoses as well as classification of idiopathic inflammatory myopathies, because they have been shown to correlate with distinct clinical phenotypes. Anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-KS, anti-OJ, anti-Ha, and anti-Zo antibodies target aminoacyl tRNA synthetases, and represent anti-synthetase syndrome...
April 2013: Brain and Nerve, Shinkei Kenkyū No Shinpo
Yoshinao Muro, Kazumitsu Sugiura, Masashi Akiyama
BACKGROUND: The myositis-specific autoantibodies that characterize certain forms of inflammatory myopathy are useful in diagnosing dermatomyositis (DM) / polymyositis and predicting its prognosis. Autoantibodies to small ubiquitin-like modifier activating enzyme (SAE) have been identified as a DM-marker antibody in European Caucasians. OBJECTIVE AND METHODS: This study investigates the frequency and clinical characteristics of anti-SAE autoantibodies in Japanese patients with DM...
June 2013: Autoimmunity
Samuel J Balin, David A Wetter, Louise K Andersen, Mark D P Davis
OBJECTIVE: To describe characteristics and treatment of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease (ACTD). DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: Seventy-eight patients with calcinosis cutis and ACTD between 1996 and 2009. MAIN OUTCOME MEASURES: Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of ACTD...
April 2012: Archives of Dermatology
Angela B Robinson, Ann M Reed
Juvenile and adult dermatomyositis (DM) have multiple commonalities, yet display differing prevalence of features, outcomes and comorbidities. In general, compared with the disease in adults, children with DM have more vasculopathy and a greater likelihood of calcinosis, periungual and gingival telangiectasias, and ulceration, but have a better long-term prognosis with improved survival. Adults with DM are more likely to have myositis-specific antibodies, develop interstitial lung disease, have amyopathic disease, and have a marked association with malignancy and other comorbidities...
September 27, 2011: Nature Reviews. Rheumatology
C Ciudad-Blanco, J A Avilés Izquierdo, M Campos-Domínguez, R Suárez-Fernández, P Lázaro Ochaita
BACKGROUND: Dermatomyositis is an idiopathic inflammatory myopathy that mainly affects the skin and skeletal muscle. An estimated 15% to 25% of patients have underlying tumors and some forms are exclusively cutaneous. The factors that predict disease course and prognosis in these patients have not been clearly identified. Here we report our experience through the description and analysis of a series of patients. MATERIAL AND METHODS: This was a retrospective study of 20 patients with a diagnosis of dermatomyositis undergoing follow-up in the Department of Dermatology at Hospital General Universitario Gregorio Marañón in Madrid, Spain between February 2007 and February 2010...
July 2011: Actas Dermo-sifiliográficas
Shinji Sato, Masataka Kuwana
PURPOSE OF REVIEW: Clinically amyopathic dermatomyositis (CADM) is a unique subset of dermatomyositis, with typical skin manifestations of dermatomyositis but little or no evidence of myositis. This review focuses on updates on epidemiology, clinical manifestations, and autoantibody profiles in patients with CADM. RECENT FINDINGS: A population-based survey of dermatomyositis conducted in the United States revealed that overall age-adjusted and sex-adjusted incidence of CADM was 2...
November 2010: Current Opinion in Rheumatology
Su-yun Ji, Fan-qin Zeng, Qing Guo, Guo-zhen Tan, Hong-feng Tang, Yi-jin Luo, Zeng-qi Tang, Yan-fang Han
BACKGROUND: Interstitial lung disease (ILD) is a serious lung complication in polymyositis (PM) and dermatomyositis (DM) which affects prognosis and requires a more aggressive approach in therapy. This study investigated the prevalence, characteristics, predictive factors and unfavourable prognostic factors of ILD in newly diagnosed PM, DM and amyopathic DM (ADM). METHODS: From January 2000 to December 2008, the medical records of 197 consecutive PM and DM patients at the Second Affiliated Hospital of Sun Yat-Sen University were reviewed excluding overlapping, juvenile, and malignancy-associated cases...
March 5, 2010: Chinese Medical Journal
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