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Childhood Dermatomyositis

Juvenile Dermatomyositis


Presentation

  • 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]
  • The length of time before the initiation of treatment affects presenting symptoms, laboratory measures, and pathophysiology.[ncbi.nlm.nih.gov]
  • Mitochondrial enzyme defect, especially CCO deficiency is present not only in genetic disorders with mitochondrial involvement but in other neuromuscular disorders including inflammatory myopathies.[ncbi.nlm.nih.gov]
  • Both fibrodysplasia ossificans progressiva (FOP) and childhood dermatomyositis with calcinosis are rare diseases, and present with ossifying or calcifying processes.[ncbi.nlm.nih.gov]
  • The clinical signs of the patients with recurrence were variable: one patient presented only heliotrope; another showed an increase in serum enzymes (without any evidence of muscular weakness) and a third patient presented intense muscular weakness and[scielo.br]
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]
  • Each child may experience 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[childrenshospital.org]
Fatigue
  • 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]
  • The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness).[childrenshospital.org]
  • 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]
  • […] 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 Sore and swollen joints Fatigue[my.clevelandclinic.org]
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]
  • 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]
  • loss joint pain and inflammation calcium deposits under the skin (called calcinosis ) mouth ulcers Symptoms may resemble other medical conditions or problems.[childrenshospital.org]
  • Other JDM signs and symptoms that may occur include: Difficulty swallowing Voice changes Muscle pain or tenderness Fatigue, fever, and weight loss Hardened deposits of calcium under the skin Stomach ulcers and intestinal tears Lung problems How is juvenile[rheumatology.org]
  • At the onset of JDM, children may have the following: Fever in the range of 101-104º Easily fatigued Less energy and doing fewer activities or just refusing to do certain tasks because they know they cannot do them successfully Weight loss and poor appetite[cincinnatichildrens.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]
Severe Clinical Course
  • clinical course variations in JDM have been identified, including the following [1, 6] : Monocyclic (remission within 2-3 y) Polycyclic (periods of remission followed by relapse) Chronic Ulcerative Extramuscular manifestations include arthritis and cardiac[emedicine.medscape.com]
Severe Abdominal Pain
  • abdominal pain (because of ulcers in the digestive system due to the vasculitis) Hardened lumps from deposits of calcium underneath the skin (calcinosis) Lung problems (because of inflammation and thickening of small airways in lungs) Due to these symptoms[my.clevelandclinic.org]
  • Your child may complain of pain with swallowing, severe abdominal pain, or bloody stools. In some cases there is very little pain but just dark or bloody stools. If any of these occur in your child, you need to contact your doctor immediately.[cincinnatichildrens.org]
  • Vasculitis of the gastrointestinal tract, observed in less than 10% of JDM patients, may result in severe abdominal pain, gastrointestinal bleeding, or perforation. 42 Abdominal pain that is persistent, progressive or severe should be carefully evaluated[obgynkey.com]
  • Alert your medical team right away if your child experiences any GI issues , in particular: Severe abdominal pain or pain that becomes progressively worse. Blood in the stool or dark, sticky stool.[curejm.org]
Increased Appetite
  • Common side effects include weight gain, increased appetite, mood changes, increased risk for infections and facial swelling.[cincinnatichildrens.org]
  • These medications all have their own side effects, but the most common ones for Prednisone are: Increased appetite and weight gain Rounded face Mood changes High blood pressure Stretch marks Fragile bones and bone damage Cataracts Slow growth In addition[curejm.org]
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.[ncbi.nlm.nih.gov]
  • The muscle weakness often causes a medical misdiagnosis of muscular dystrophy or other muscle disease.[en.wikipedia.org]
Myopathy
  • 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]
  • Mitochondrial enzyme defect, especially CCO deficiency is present not only in genetic disorders with mitochondrial involvement but in other neuromuscular disorders including inflammatory myopathies.[ncbi.nlm.nih.gov]
  • Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy (IMM) of presumed autoimmune dysfunction resulting in muscle weakness among other complications.[en.wikipedia.org]
  • 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]
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.[ncbi.nlm.nih.gov]
  • Similar to adult cases of dermatomyositis, the disease is primarily characterized by progressive, symmetrical, proximal muscle weakness.[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]
  • 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]
  • 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]
  • 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]
Proximal Muscle Weakness of the Lower Extremity
  • Proximal muscle weakness (upper or lower extremity and trunk) 3. Elevated serum creatine kinase or aldolase level 4. Muscle pain on grasping or spontaneous pain 5.[aafp.org]
Malar Rash
  • About 40% of children also have a prominent erythematous malar rash. This rash can be ulcerative, cross the nasolabial folds, and extend onto the forehead.[clinicalgate.com]
  • This child also has a malar rash.[obgynkey.com]
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

  • 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]
  • The best predictors of good functional recovery and minimal calcinosis were early treatment after the onset of symptoms and low creatine kinase serum level at the time of diagnosis.[ncbi.nlm.nih.gov]
  • 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]

Prognosis

  • The technique of nailfold capillaroscopy may prove to be a clinically useful, noninvasive means of early prognosis.[ncbi.nlm.nih.gov]
  • Modern treatment of childhood dermatomyositis with corticosteroids has resulted in greatly improved prognosis and style of life.[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]

Etiology

  • 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]
  • 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]
  • 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]
  • It is a systemic disorder of unknown etiology, characterized by necrosis and regeneration of muscle fibers, with inflammation of muscle, skin and other tissues.[scielo.br]
  • Although the etiology of juvenile DM remains unclear, it is suggested that IgE can be associated with autoimmune diseases, such as systemic lupus erythematosus (SLE) and juvenile DM, by mediating the release of chemical mediators and by faciliating the[kjim.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • For patient education, parents and patients may visit the following Web sites: Pathophysiology The current model of the pathogenesis of JDM involves both humoral and cell-mediated mechanisms that cause vascular and muscle damage.[emedicine.medscape.com]

Prevention

  • 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]
  • 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]
  • Also, there is nothing you could have done to prevent your child from developing this disease.[cincinnatichildrens.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]

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