Juvenile Dermatomyositis Clinical Presentation

Updated: May 12, 2021
  • Author: Ann M Reed, MD; Chief Editor: Lawrence K Jung, MD  more...
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Presentation

History

Constitutional, respiratory, and GI symptoms may occur within 3 months of onset of juvenile dermatomyositis (JDM). [57] With eruption of skin lesions, pruritus may be present in 38% of children. [69] Photosensitive rashes may occur. [2] Muscle involvement can be insidious, with development of functional limitations such as difficulty getting out of bed or tiring easily from sporting events. [32]

Other common symptoms include fever, dysphagia, dysphonia or hoarseness, myalgias, arthralgias, abdominal pain, and melena from GI involvement as a consequence of vasculopathy. [32, 69]

Rarely, pneumatosis intestinalis or colonic perforation may occur. [70, 71]

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Physical Examination

JDM primarily affects the skin and muscles. The eyelids and face may be swollen with a heliotrope rash (the color of a garden perennial), a purple or dusky mauve color in the periorbital region, and an overlying scale. Periorbital edema is sometimes present. See image below.

A characteristic, violaceous rash is present over A characteristic, violaceous rash is present over the eyelids with periorbital edema in a child with juvenile dermatomyositis.

A malar rash in a photosensitive distribution with sparing of the nasolabial folds may occur, making the diagnosis of JDM difficult to distinguish from systemic lupus erythematosus. [72]

Gottron papules are shiny, elevated, violaceous papules and plaques present over the bony prominences such the metacarpophalangeal joints, the proximal interphalangeal joints, the distal interphalangeal joints, the elbows, the knees, and the ankles. Sparing of the interphalangeal spaces is observed. See image below.

Gottron papules are present over the metacarpophal Gottron papules are present over the metacarpophalangeal joints and proximal interphalangeal joints in a child with juvenile dermatomyositis.

Erythematous, violaceous scaly plaques may occur on the extensor surfaces of the extremities (see the image below).

An erythematous, violaceous, scaly rash is present An erythematous, violaceous, scaly rash is present over extensor surfaces in a child with juvenile dermatomyositis.

Nailfold telangiectasias, periungual erythema, poikiloderma, lichenification, and psoriasiform dermatitis may be seen. Hypertrophic, ragged cuticles may accompany periungual erythema. [69] Inadequately treated children have persistent nailfold abnormalities reflective of skin disease activity but not muscle involvement. [73]

Diffuse vasculopathy may be associated with vasomotor instability, such as Raynaud phenomenon, livedo reticularis, or vascular infarctions on the medial canthus of the eyelids.

Mechanic's hands may occur, with hyperkeratosis and peeling of the skin over the lateral and palmar aspects of the fingers. Mechanic’s hands are usually seen in the setting of myositis-specific autoantibodies and interstitial lung disease. [74]

Less common findings related to complement deposition and associated with a more severe disease course include cutaneous and mucosal ulcerations. [7, 14, 69]

Symmetrical proximal muscle weakness involving the deltoids, quadriceps, or both is a prominent clinical finding in JDM. [69] These children may demonstrate a Gower maneuver (ie, needing to use their arms to raise themselves from the floor to a standing position).

Calcinosis cutis

Although calcinosis (a manifestation of dystrophic calcification with normal serum calcium and phosphorus levels) is rare in adults with dermatomyositis, it occurs in 20-40% of patients with JDM. [64] The deposits are firm, white or flesh-colored nodules over bony prominences that have a high mineral content of calcium hydroxyapatite, as well as osteopontin, osteonectin, and bone sialoprotein (see the image below). [75]

Calcinosis cutis over the left elbow in a patient Calcinosis cutis over the left elbow in a patient with juvenile dermatomyositis for 16 years.

The sites most commonly affected with calcinosis cutis include the elbows, knees, and extremities. Onset is within 3 years of diagnosis but may occur as long as 20 years later. [76, 64]

Calcinosis is divided into the following 4 subtypes [77] :

  • Superficial calcareal masses

  • Deep calcareal masses

  • Linear deposits

  • Subcutaneous deposition of calcium that encase the torso

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