Dermatomyositis: Symptoms, Complaints, Signs

The following symptoms and complaints may indicate dermatomyositis (muscle inflammation with skin involvement):

Main symptoms

Skin involvement:

  • Head/face
    • Alopecia (hair loss)
    • Scaling in sun-exposed areas (forehead, auricles, walls and neck (shawl sign).
    • Erythema (skin redness), slightly purple in color – this extends to the parts of the body exposed to sunlight (scalp, face, neck, neckline, upper part of the back, upper limbs) (= heliotropic erythema)
      • Among other things, heliotropic (purple) erythema of the eyelids.
    • Edema of the periorbital region (swelling lateral and below the orbit), also possible on the forehead and cheeks.
    • Facial expression: tearful (hypomimia).
  • Extremities
    • Finger and metacarpophalangeal joints: symmetrically occurring Gottron papules (papules (nodular thickening of the skin) with a smooth surface and livid discoloration on the finger extensor sides) [pathognomonic sign, i.e., “clearly indicative of disease”]
    • Atrophy (tissue atrophy)
    • Erythema with scaling
    • Rough and cracked fingers (“mechanic’s hands”)
    • Teleangiectasia (vascular veins)
  • Nails
    • Hyperkeratosis (“thickening”) of the cuticle.
    • Nail fold changes
    • Thickening of the cuticle (so-called Keining sign).
    • Teleangiectasia and splinter hemorrhages in the area of the nail fold.
  • Trunk
    • Atrophy (tissue loss)
    • Hyper-/Depigmentation
    • Poikilodermic foci (“multicolored skin”)
    • Teleangiectasias (vascular veins)

Musculature related:

  • Symmetrical muscle weakness (especially proximal extremity muscles/upper arms and thighs or shoulder/pelvic girdle).
  • Muscle soreness myalgias (muscle pain).
  • Sclerosis (hardening) and atrophy of shoulder/upper arm and pelvic/thigh muscles.
  • Affected individuals are unable to raise their arms above their head and/or have difficulty climbing stairs, standing up.
  • Notice: In the early stages of the disease, muscle involvement is often absent (pure amyopathic form) or more often clinically silent (clinical amyopathic DM).

Secondary symptoms

  • Fatigue
  • Fever
  • Joint pain

Involvement of internal organs is possible:

  • Esophagus (esophagus): dysphagia – in 30% of cases.
  • Heart: interstitial myocarditis (inflammation of the heart muscle) – in 30% of cases; tachycardia (heartbeat too fast: > 100 beats per minute) and changes in the ECG are possible.
  • Lungs: alveolitis (disease of the lung tissue and alveoli (alveoli)), fibrosis (pathological increase in connective tissue of the lungs) – in 30% of cases.

Typical constellation of symptoms sufficient for a tentative diagnosis:

  • Lilac/purple erythema (redness of the skin).
  • Reduction in strength
  • Myalgia (muscle pain)