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Juvenile dermatomyositis in Child
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Juvenile dermatomyositis in Child

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Contributors: Kyle Cheng MD, Susan Burgin MD, Craig N. Burkhart MD, Dean Morrell MD
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This summary discusses dermatomyositis in children. Dermatomyositis in adults is addressed separately.

Childhood type dermatomyositis, also known as juvenile dermatomyositis (JDM), is an idiopathic multisystem inflammatory disorder where muscle inflammation is accompanied by a skin rash. It is typically first detected in children aged between 2 and 15 years.

Cutaneous lesions may precede muscle disease by anywhere from weeks to more than 4 years and may be extremely pruritic. Exposure to sunlight may exacerbate the cutaneous lesions. Other characteristic features may include flat erythema of the upper back and posterior neck and shoulders (shawl sign) as well as a similarly presenting macular erythema of the anterior neck and upper chest (V sign) that can worsen with ultraviolet (UV) exposure. Patients may also have poikiloderma over the lateral hip (holster sign). Myositis (inflammation of muscle) usually develops insidiously, but sometimes abruptly, or may not occur at all (amyopathic dermatomyositis). Muscle findings include symmetric proximal muscle weakness, usually of the shoulder and pelvic girdles.

In acute phases, pain and tenderness may occur and palpation may have a doughy feel. In addition, the patient's gait may be abnormal. Patients may complain of difficulty climbing stairs and brushing their hair. Upper palatal muscle and esophageal dysfunction may be noted. Neck flexors are usually weak, and the patient may be unable to raise his or her head at all.

While children experience calcinosis cutis, muscle necrosis, and gingival and periungual telangiectasias more frequently than adults, JDM is a milder disease overall than adult dermatomyositis. There is a lower frequency of interstitial lung disease and lower overall mortality. Moreover, the association of malignancy with dermatomyositis is extremely uncommon in JDM.

Signs of severity in childhood include gastrointestinal tract involvement, high severity of muscle disease, and endomysial fibrosis on muscle biopsy. The presence of these signs may indicate disease that is refractory to first-line treatment.

In 2017, the European League Against Rheumatism / American College of Rheumatology (EULAR/ACR) released new classification criteria for idiopathic inflammatory myopathies.


M33.90 – Dermatopolymyositis, unspecified, organ involvement unspecified

1212005 – Juvenile dermatomyositis

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The most underdiagnosed form of the disease is the eruption without muscle weakness.

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Last Updated: 02/05/2018
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Juvenile dermatomyositis in Child
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Juvenile dermatomyositis : Dorsum of hand, Elbow, Eyelid edema, Face, Fingers, Heliotrope sign, Muscle weakness, Nail fold telangiectasia, Photosensitivity, Sun-exposed distribution, Smooth papules, WBC decreased, CK increased
Clinical image of Juvenile dermatomyositis
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