Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Mamyrova, Gulnara; Kishi, Takayuki; Targoff, Ira N.; Ehrlich, Alison; Curiel, Rodolfo V.; Rider, Lisa G.

doi:10.1093/rheumatology/key190

Cited by 28 publications

(44 citation statements)

References 27 publications

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“…Juvenile dermatomyositis (JDM) is the most common myopathy in childhood . It is a vasculopathy which is associated with proximal muscle weakness and characteristic skin manifestations such as microvascular dilation, Gottron's papules over extensor joint surfaces, and a heliotrope rash around the eyes . The etiology of the disease remains unknown.…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of the disease remains unknown. Juvenile dermatomyositis may present as an amyopathic as well as a myopathic variant . Characteristic features of JDM according to the latter survey are proximal muscle weakness (100%), arthralgia (55%), Gottron's papules (93%), heliotrope rash (87%), photosensitivity (55%), fatigue (82%), weight loss (40%), and abdominal pain (36%) .…”

Section: Discussionmentioning

confidence: 99%

“…Juvenile dermatomyositis may present as an amyopathic as well as a myopathic variant . Characteristic features of JDM according to the latter survey are proximal muscle weakness (100%), arthralgia (55%), Gottron's papules (93%), heliotrope rash (87%), photosensitivity (55%), fatigue (82%), weight loss (40%), and abdominal pain (36%) . In clinically amyopathic JDM (CAJDM) myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain, fatigue, and blood and serum abnormalities are less frequent.…”

Section: Discussionmentioning

confidence: 99%

“…In clinically amyopathic JDM (CAJDM) myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain, fatigue, and blood and serum abnormalities are less frequent. However, CAJDM and classic juvenile dermatomyositis (JDM) share a high percentage of positive ANA titers (70%‐80%) …”

Section: Discussionmentioning

confidence: 99%

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Erythematous scaling lesions of the face, dorsal fingers, elbows, and knees together with symmetrical muscle weakness in a child

Markovic

Schuster²,

Simon

et al. 2019

Clinical Case Reports

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Erythematous scaling lesions of the face, dorsal fingers, elbows, and knees together with symmetrical muscle weakness in a child

Markovic

Schuster²,

Simon

et al. 2019

Clinical Case Reports

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“…Завершуючи короткий огляд перебігу ЮДМ без ураження мускулатури та порівнюючи його з отриманими нами даними, відкритим залиша ється важливе питання: враховуючи все таки наявність у пацієнта стійкого, здатного до рецидиву, запально некротичного ураження язика, то -це ЮДМ аміопатичний, ЮДМ гіпо аміопатичний (враховуючи підвищену актив ність АЛТ у дебюті захворювання [21,32]), чи це якийсь інший фенотип хвороби? З деякою аналогією в цьому сенсі можна розглядати повідомлення норвезьких ревматологів та кар діологів про ізольоване порушення зі сторони іншого поперечно посмугованого м'язового органу -серця: у 59 пацієнтів з тривалістю хвороби від 2 ох до 38 ми років (медіана спостереження -16,8 років) та високою актив ністю на її початку з подальшим переважанням шкірних уражень без м'язової участі, виявлено достовірно більшу, у порівнянні з контрольною групою, кількість осіб із ознаками систолічної та діастолічної дисфункції міокарда лівого шлуночка [39].…”

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Debut and course rare of juvenile dermatomyositis. Part II: clinical analysis with comparative analytical review of the literature

Rеitmаier¹,

Volosyanko²,

Synoverska³

et al. 2020

MPU

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The first part of the article describes a rare case of the onset and course of juvenile dermatomyositis. In the second part the comparative clinical analysis of results of own supervision with other similar reports is carried out. The main attention is focused on atypical variants of the onset of the disease, in particular signs of severe intoxication syndrome, hemorrhagic-necrotic rash, anasarca, lesions of the oral cavity in the form of stomatitis and ulcerative-necrotic glossitis. The difficulties of early diagnosis of the disease on the background of delayed manifestation of pathognomonic skin signs and the absence of reliable features of myopathic syndrome are shown. Clinical and laboratory characteristics of amyopathic juvenile dermatomyositis with persistent skin lesions have been defined. There are three differentiated plans-recommendations of the Childhood Arthritis and Rheumatology Research Alliance for the treatment of patients who have never had muscle damage and those who have had clinical manifestations of myopathy only in the first months of the disease. Current trends in achievements and gaps in the classification, diagnosis and treatment of various forms of juvenile dermatomyositis have been observed. On the basis of scientific publications the possible prognostic consequences of the amyopathic form of this disease in children are highlighted. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: juvenile dermatomyositis, juvenile amyopathic dermatomyositis, atypical course, ulcerative necrotic glossitis, anasarca, psoriasis, treatment.

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Clinically Amyopathic Juvenile Dermatomyositis

Shawa

Cotter

2022

JAMA Dermatol

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An otherwise healthy 6-year-old girl presented with 4 years of a rash on her hands, feet, elbows, and knees. Previously, she had used treatment with wet wraps and topical steroids without improvement. Her review of systems was notable for rare cough and dyspnea, but negative for muscle weakness. Physical examination revealed ragged cuticles; flat-topped pink papules overlying the bilateral metacarpophalangeal, proximal interphalangeal, and distal interphalangeal joints that were consistent with Gottron papules; and a spontaneous skin ulceration on her right knee (Figure). Her muscle strength was normal. Biopsy results of a metacarpophalangeal lesion revealed a lichenoid tissue reaction with basal vacuolation, which was consistent with dermatomyositis. Laboratory study results were notable for an antinuclear antibody titer of 1:320. Aldolase, creatine kinase, and myositis antibody panel results (Quest Diagnostics) were normal. Given the clinicopathologic features, a diagnosis of clinically amyopathic juvenile dermatomyositis (JDM) (CAJDM) was made. Treatment with hydroxychloroquine, prednisone, and clobetasol was administered while care was coordinated with pediatric pulmonology, rheumatology, and physical therapy departments. Ultimately, she was transitioned to receive treatment with methotrexate and intravenous immunoglobulin. At 1-year followup, myositis and interstitial lung disease remained absent and skin findings had improved.Clinically amyopathic dermatomyositis (CADM) is a rare variant of dermatomyositis that may present with some or all of the classic cutaneous findings of dermatomyositis. Heliotrope rash on the upper eyelids is the most common finding. Gottron papules involving the extensor surfaces are pathognomonic. Other findings include periungual telangiectasias, scalp involvement, nonscarring alopecia, and poikiloderma. As suggested by the name, CADM lacks muscular involvement; however, interstitial lung disease, myocarditis, scleroderma, and cancer remain associated with this variant.African American individuals exhibit greater risk of developing dermatomyositis, and African American children with JDM are more likely to develop calcinosis and vasculitis. 1 In contrast, pediatric patients without myopathy have lower rates of vasculopathy, interstitial lung disease, and calcinosis. The average age of diagnosis of CAJDM is 11 years. While some patients may develop classic dermatomyositis, most do not develop myopathy.

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Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Abstract: CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.

Cited by 28 publications

References 27 publications

Erythematous scaling lesions of the face, dorsal fingers, elbows, and knees together with symmetrical muscle weakness in a child

Erythematous scaling lesions of the face, dorsal fingers, elbows, and knees together with symmetrical muscle weakness in a child

Debut and course rare of juvenile dermatomyositis. Part II: clinical analysis with comparative analytical review of the literature

Clinically Amyopathic Juvenile Dermatomyositis

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