A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

Some patients can present calcinosis3 and the gastrointestinal tract, kidney and myocardium can be involve4,5,6. [scielo.br]

The juvenile IIM also may be classified based on the presence of certain autoantibodies that are present specifically in myositis patients (myositis-specific autoantibodies), and others that are present in myositis patients as well as patients with other [obgynkey.com]

Part IV (Overview) summarizes the issues that may not have been clear and highlights differing and unsettled views or present available data. [books.google.de]

• Fever

  The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness). [childrenshospital.org]

  […] a) fluctuation of the rash with twice daily fevers b) blue color changes to the hands with cold exposure c) oral ulcers d) new difficulty keeping up with peers in soccer Answer 2. [pediatrics.mc.vanderbilt.edu]

  […] the neck, shoulders, trunk, back or hips) Difficulty with everyday activities, such as climbing stairs, standing up, riding a bicycle, getting dressed, lifting the head Frequently falling down Weak voice (dysphonia) Difficulty swallowing ( dysphagia ) Fever [my.clevelandclinic.org]

• Fatigue

  The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness). [childrenshospital.org]

  When a child becomes irritable, fatigued, reluctant to socialize, and the face becomes flushed easily, physicians refer to this constellation of symptoms as "misery." The speed of the progression of JDMS is highly variable. [en.wikipedia.org]

  The inflammation of the muscles results into weakness of the muscles, clumsiness, fatigue, and even physical inactivity. [web.archive.org]

  Fatigue and fatigue along with muscle weakness are common symptoms Muscle pain may occur, but not at all Thigh muscles are most commonly attacked Symptoms may be trouble getting up from the floor or the chair, climbing stairs and hills Skin symptoms are [bindevevssykdommer.no]

  In addition to the classical symptoms of proximal symmetric muscle weakness further symptoms such as arthralgia, fatigue, and dysphagia can occur in DM. [thieme-connect.com]

• Weight Loss

  We describe a case of juvenile dermatomyositis associated with hepatocarcinoma.A 14-year-old boy with a weight loss of 15 kg over a three-month period presented with clinical signs of dermatomyositis, including typical edematous rash, muscular weakness [nejm.org]

  Dysphagia, myalgia, fever and weight loss are other features 7. There is cell-mediated injury targeted at striated muscle with resultant atrophy, edema, coagulation necrosis, fibrosis and calcification. Markers elevated muscle enzymes (e.g. [radiopaedia.org]

  Constitutional symptoms or signs, as used in these listings, means severe fatigue, fever, malaise, or involuntary weight loss. [ssa.gov]

  About half of children with JDMS also have pain in their muscles.Other symptoms may include irritability, weight loss, and mouth ulcers. [en.wikipedia.org]

  If the muscles in your esophagus are affected, you can have problems swallowing (dysphagia), which can cause weight loss and malnutrition. Aspiration pneumonia. [mayoclinic.org]

• Malaise

  A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

  […] symptoms differently, but the most common signs are: fever rash around the eyelids (known as heliotrope rash ) and/or knuckles and finger joints (called Gottren’s papules ); a rash may also occur on the elbows, knees and ankles muscle weakness fatigue malaise [childrenshospital.org]

  Some individuals with dermatomyositis may also develop symptoms such as low-grade fever, a general feeling of ill health (malaise), shortness of breath (dyspnea), pain in multiple joints (polyarthralgia), weight loss, and/or other abnormalities. [rarediseases.org]

  Some patients display constitutional symptoms including fever, weight loss, anorexia, and malaise. [rheumaknowledgy.com]

• Anorexia

  Prognosis Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis. [aafp.org]

  Some patients display constitutional symptoms including fever, weight loss, anorexia, and malaise. [rheumaknowledgy.com]

• Hoarseness

  Palatal involvement leading to hoarseness and dysphonia is seen in up to 45% of patients. 31,33 Respiratory muscle weakness with dyspnea on exertion and restrictive lung disease is common, 44 but interstitial lung disease is relatively infrequent in juvenile [obgynkey.com]

  Coughing or choking while eating stomach discomfort or pain Heartburn, indigestion, acid reflux Slow stomach emptying (gastroparesis) Chronic diarrhea or constipation Silent aspiration (where the voice sounds gurgly or wet after swallowing) Voice changes (hoarseness [curejm.org]

  Your child's voice may also sound faint or hoarse. If you notice these symptoms happening, contact the doctor immediately. Stiff and Swollen Joints Your child may complain of stiff and sore joints. [cincinnatichildrens.org]

• Dysphagia

  Dysphagia, myositis-specific or -related antibodies, factor VIII-related (von Willebrand) antigen, myalgia, muscle ultrasound and ulcerations had a median score of 4. [doi.org]

  The signs could include dysphonia, falling or dysphagia. Due to these symptoms, JDM is often misdiagnosed as muscular dystrophy or any other disease of the muscle. A few patients develop contractures (the muscle retracts and the joints remain bent). [web.archive.org]

  Five children developed dysphagia necessitating tube feeding and more aggressive therapy. Dysphagia due to weakness of the pharyngeal musculature is associated with severe forms of JDM. [indianpediatrics.net]

  In addition to the classical symptoms of proximal symmetric muscle weakness further symptoms such as arthralgia, fatigue, and dysphagia can occur in DM. [thieme-connect.com]

• Oral Ulcers

  […] a) fluctuation of the rash with twice daily fevers b) blue color changes to the hands with cold exposure c) oral ulcers d) new difficulty keeping up with peers in soccer Answer 2. [pediatrics.mc.vanderbilt.edu]

  Lymphoma, nausea, vomiting, hepatotoxicity, leukopenia, oral ulcers, thrombocytopenia Screen patients for thiopurine methyltransferase deficiency before therapy (0.3 to 11 percent of white population). [aafp.org]

• Muscle Weakness

  A muscle biopsy was inconclusive, however, she was treated for a presumptive diagnosis of inflammatory myositis. In a second flare in childhood, she developed proximal muscle weakness and calcinosis cutis. [ncbi.nlm.nih.gov]

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  The muscle weakness often causes a medical misdiagnosis of muscular dystrophy or other muscle disease. [en.wikipedia.org]

• Myopathy

  […] with lung involvement Juvenile dermatopolymyositis with myopathy Clinical Information A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. [icd9data.com]

  New perspectives on the idio-pathic inflammatory myopathies of childhood. Curr Opin Rheumatol 1994; 6(6):575–582 PubMed CrossRef Google Scholar Rider LG. Outcome assessment in the adult and juvenile idiopathic inflam-matory myopathies. [link.springer.com]

  Mononuclear cells in myopathies: quantification of functionally distinct subsets, recognition of antigen-specific cell-mediated cytotoxicity in some diseases, and implications for the pathogenesis of the different inflammatory myopathies. [scielo.br]

  Juvenile dermatomyositis, the most common inflammatory myopathy of childhood, is a rare systemic autoimmune vasculopathy that is characterised by weakness in proximal muscles and pathognomonic skin rashes. [ncbi.nlm.nih.gov]

• Arthritis

  Arthritis Rheum 2000; 43(3):541–549 PubMed CrossRef Google Scholar Lindehammar H, Lindvall B. [link.springer.com]

  US incidence of juvenile dermatomyositis, 1995-1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases Registry. Arthritis Rheum. 2003 Jun 15. 49(3):300-5. [Medline]. [emedicine.medscape.com]

  Juvenile idiopathic arthritis disease-specific research Committee of Childhood Arthritis Rheumatology and Research Alliance. Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis. [ped-rheum.biomedcentral.com]

  Like many inflammatory arthritis diseases, early aggressive treatment is vitally important. [jointhealth.org]

  Documentation of inflammatory arthritis. [ssa.gov]

• Proximal Muscle Weakness

  A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  He considers that this may be dermatomyositis and tests for proximal muscle weakness. The proximal muscles are not found to be weak on physical exam although a subsequent blood test showed a mildly elevated CK and AST. [mhmedical.com]

• Myalgia

  Dysphagia, myositis-specific or -related antibodies, factor VIII-related (von Willebrand) antigen, myalgia, muscle ultrasound and ulcerations had a median score of 4. [doi.org]

  Dysphagia, myalgia, fever and weight loss are other features 7. There is cell-mediated injury targeted at striated muscle with resultant atrophy, edema, coagulation necrosis, fibrosis and calcification. Markers elevated muscle enzymes (e.g. [radiopaedia.org]

  Fever, weight loss, fatigue, myalgias, arthritis, lymphadenopathy, and abdominal pain are constitutional symptoms known to be associated with JDM. [5], [7] Gastrointestinal system involvement may lead to gut vasculitis and perforation; similarly, dyspnea [ijpd.in]

  Less than half of patients complain of myalgia at the onset. Pharyngeal skeletal muscle may be involved, causing upper pharyngeal dysphagia, dysphonia, nasal regurgitation, and the risk of aspiration. [rheumaknowledgy.com]

• Ulcer

  Ulcers The ulcers in JDM are caused by breakdown of the tissue surrounding an involved blood vessel that is not providing enough circulation to the tissue. These ulcers most commonly occur in the skin and gastrointestinal tract. [cincinnatichildrens.org]

  Dysphagia, myositis-specific or -related antibodies, factor VIII-related (von Willebrand) antigen, myalgia, muscle ultrasound and ulcerations had a median score of 4. [doi.org]

  Skin ulcerations reflect significant vasculopathy of the skin and may be a sign of internal organ vasculopathy. Ulcerative skin lesions are associated with more severe disease and worse prognosis. [clinicalgate.com]

  By a retrospective analysis of the childhood dermatomyositis patients, we found that the presence of nailfold capillary abnormalities correlates with more severe forms of the disease (ulcerative and chronic types), as opposed to limited type of disease [ncbi.nlm.nih.gov]

• Skin Rash

  All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. [doi.org]

  The skin rash generally starts in the first few weeks after the onset of muscle symptoms. However, the skin rash may be absent early on and many physicians may fail to recognize this disease during its early stages. [cincinnatichildrens.org]

  The main symptoms of juvenile dermatomyositis are muscle weakness and skin rashes. This is caused by inflammation in the small blood vessels ( vasculitis ) in skin and muscle and also by inflammation of the muscle cells. [my.clevelandclinic.org]

• Eczema

  The rash that is found on the face and hands most often resemble allergies, fifth disease, eczema or other more common skin symptoms. The color is what sets the JDM apart from all these other symptoms, though. [web.archive.org]

  Gottron’s papules are frequently confused with severe eczema if JDMS is not suspected. [clinicalgate.com]

  (This may be mistaken for eczema .) [my.clevelandclinic.org]

  On the hands and face, the rash very closely resembles allergies, eczema, fifth disease, or other more common skin condition, but the heliotrope color is unique to the inflammatory process of JDMS. [en.wikipedia.org]

  Often, the characteristic skin rashes are first misdiagnosed as eczema or psoriasis. Muscle pain can be mistaken for growing pains or overuse, and weakness can be confused with laziness or sleepiness. [jointhealth.org]

• Skin Ulcer

  […] biopsy and skin ulcerations. [doi.org]

  Half a year later, she experienced skin ulceration and papules around the joints. Physical examination revealed violaceous rashes over the eyelids [Figure 2] a. [ijdvl.com]

  Skin ulcerations reflect significant vasculopathy of the skin and may be a sign of internal organ vasculopathy. Ulcerative skin lesions are associated with more severe disease and worse prognosis. [clinicalgate.com]

  Desquamating dermatitis with splitting of the skin may evolve over the radial aspects of the fingers. Subcutaneous calcification may occur, particularly in children. Photosensitivity and skin ulceration are visible in dermatomyositis as well. [merckmanuals.com]

• Flushing

  When a child becomes irritable, fatigued, reluctant to socialize, and the face becomes flushed easily, physicians refer to this constellation of symptoms as "misery." The speed of the progression of JDMS is highly variable. [en.wikipedia.org]

  […] of etanercept but did not return for the 24-week visit, and 1 patient stopped the study after the fifth dose of etanercept due to marked worsening of a rash that was diffuse (shawl sign, extremities), increased over joints, and had an intense malar flush [onlinelibrary.wiley.com]

• Malar Rash

  The classic heliotrope rash is a violaceous discoloration of the eyelids with associated eyelid edema; this is present in approximately 80% of children at diagnosis. About 40% of children also have a prominent erythematous malar rash. [clinicalgate.com]

  Malar and facial erythema, and erythematous rashes involving both sun-exposed and covered areas, are also common. [obgynkey.com]

• Lethargy

  A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. [ncbi.nlm.nih.gov]

  Systemic manifestations such as fever, lethargy and general discomfort may be the first symptoms of the illness, with minimal muscle weakness. Cutaneous lesions are frequent and they appear at the onset or during the course of the disease. [scielo.br]

  Patients most commonly present with lethargy, pruritus, fatigue, photosensitivity or arthralgias. [physio-pedia.com]

  This condition has been reported in approximately 2 to 11 percent of patients with dermatomyositis. 7, 21, 23 Patients most commonly present with lethargy, pruritus, fatigue, photosensitivity or arthralgias. 8 In some cases, myositis developed later; [aafp.org]

• Asthenia

  Prognosis Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis. [aafp.org]

• Unable to Walk

  Sometimes muscle symptoms never appear at all or occur very gradually over the course of months, and sometimes going from normal strength to being unable to walk within days. [en.wikipedia.org]

Treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents. Disease outcome, if treatment is initiated early, is generally good. [ncbi.nlm.nih.gov]

To reach these goals, doctors work with families to find a treatment plan that works best for them. Treatment usually includes medication, physical therapy, and education. As your child's symptoms change, the treatment plan may also be changed. [cincinnatichildrens.org]

Diet is also an important component of treatment for any disease, and this is especially true for one requiring steroid treatment. [jointhealth.org]

Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity [journals.lww.com]

The technique of nailfold capillaroscopy may prove to be a clinically useful, noninvasive means of early prognosis. [ncbi.nlm.nih.gov]

Ulcerative skin lesions are associated with more severe disease and worse prognosis. Buy Membership for Pediatrics Category to continue reading. Learn more here Netters Pediatrics [clinicalgate.com]

Differential diagnosis is important because treatment and prognosis is different. [kjim.org]

Prior to the widespread use of corticosteroids, the prognosis was poor. [emedicine.medscape.com]

Part II (Etiology and Mechanisms) covers advances in the immunopathology and viral etiology of PM/DM along with a frequently recognized entity: inclusion body myositis. [books.google.de]

Published on May 23, 2012 Dermatomiositis juvenil Abstract 13 Background: Juvenile dermatomyositis is a multisystemic disease of unknown etiology, characterized by non-suppurated inflamma- t... [issuu.com]

The etiology of jDM is unknown and the underlying immune mechanism poorly understood which is why current studies address the question of environmental triggers, genetic predisposition, or maternal microchimerism. [thieme-connect.com]

In patients with overlap syndrome, the myositis tends to respond better to treatment with corticosteroids than it does in patients with an idiopathic etiology. [physio-pedia.com]

Genetic factors and environmental triggers such as enterovirus and group B streptococcus infection and ultraviolet radiation exposure are likely to play an important role in the etiology. [2] JDM has been associated with significant mortality and morbidity [ijpd.in]

Understanding the epidemiology and treatment outcomes of this disease in children is important for better planning of appropriate diagnostic and treatment interventions. [ajol.info]

Clinical Classification of the Juvenile Idiopathic Inflammatory Myopathies (IIM) EPIDEMIOLOGY The annual incidence of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) ranges from 1.5 to 5 cases per 1 million children in various countries [obgynkey.com]

The epidemiology of polymyositis. Am J Med 1970; 48: 715-23. [ Links ] 2. Bridges BF. The rashes of dermatomyositis in a black patient. Am J Med 1991; 91: 661-2. [ Links ] 3. Pachman LM. Juvenile dermatomyositis. [scielo.br]

Further research examining the mechanisms of action of UVR in the pathogenesis of juvenile IIM is needed. [52, 53] Epidemiology In the United States, the annual incidence of JDM ranges from 2.5-4.1 cases per million population. [54] In the United Kingdom [emedicine.medscape.com]

Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am. 2002;28(4):723-41. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. [malattierare.regione.veneto.it]

The length of time before the initiation of treatment affects presenting symptoms, laboratory measures, and pathophysiology. [ncbi.nlm.nih.gov]

GENETICS AND PATHOPHYSIOLOGY The pathogenesis of JDM appears to involve chronic immune activation in genetically susceptible individuals following exposure to specific environmental triggers. [obgynkey.com]

Environmental and genetic factors affect the child's susceptibility to these conditions and also alter the inflammatory response, adding heterogeneity to disease pathophysiology. [link.springer.com]

Further understanding of the pathophysiology, role of cytokines in inflammatory myopathies, and pharmacogenomics should guide future clinical trials in patients with juvenile DM. [onlinelibrary.wiley.com]

In contrast, the main pathophysiologic abnormality in polymyositis is direct T cell-mediated muscle injury. [merckmanuals.com]

Delay in the diagnosis of FOP is common, although early recognition of FOP prevents a child from accidental or iatrogenic injury, which can precipitate ectopic ossification. [ncbi.nlm.nih.gov]

Early diagnosis and sticking to the treatment plan are important to prevent permanent muscle weakness. Children experience JDM differently. [rheumatology.org]

Physical therapy is directed at preventing muscle wasting, stiffness and contractures. Speech therapy. If the throat muscles become weak, speech therapy can help improve speech and swallowing. Skin protection. [arthritis.org]

Children with juvenile dermatomyositis should eat a diet low in sodium and high in calcium, to help prevent the brittle bones that are a potential side-effect of steroid treatment. [jointhealth.org]

Frequent exercise can prevent contractures. The first muscles that are first to be affected tend to be the proximal muscles such as those that are located on the shoulders, neck, back and abdomen. [web.archive.org]