Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 4A41.0Z

  • Wagner 1863
  • Unverricht 1887


Rare antibody- or immune complex-mediated autoimmune disease, which belongs to the group of inflammatory myopathies.

  • Incidence (children): 2-7/1'000'000/year
  • Incidence (adults): 6-10/1'000'000/year
  • Peak of incidence (adults): ca. 40 LY and v. 55-60 yrs
  • Peak in incidence (children): 5-14 yrs
  • African-Americans > Caucasians
  • Women : men = up to 2 : 1

  • Clinical are distinguished (with frequencies):
    • Type I: adult polymyositis
    • Type II: typical adult dermatomyositis with skin/muscle involvement
    • Type III: typical adult dermatomyositis with malignant tumours
    • Type IV: Typical dermatomyositis of the adult of childhood (especially after infection of the respiratory tract)
    • Type V: Polymyositis with myoglobulinuria (acute myolysis)
    • Type VI: Polymyositis with Sjörgren's syndrome
    • Type VII: Polymyositis with other collagenoses

  • UV light may lead to exacerbation of dermatomyositis
  • Genetic predispositions:
    • Idiopathic myositis: HLA-B8, HLA DRB 03
    • Classic and juvenile dermatomyositis: HLA-A68, HLA-DR3
    • Drug-associated dermatomyositis: HLA-B 08, HLA-DR4 haplotypes
    • Amyopahtic dermatomyositis: no HLA associations
  • Infections:
    • Picornaviruses
    • Coxsackieviruses
    • AIDS
    • HTLV-1
  • Medications:
    • D-penicillamine
    • Procainamide
    • NSAR
    • Simvastatin
    • Antimalarials
    • Interferon α
    • Allopurinol
  • Neoplasia

  • General symptoms
  • Fatigue
  • Myalgias (muscle-cartilage-like)
  • Problems combing hair and other daily activities


  • Cutaneous symptoms:
    • Facial swelling with eyelid oedema
    • Pruriginous or mildly dolente, lilac erythema on the face and décolleté
    • "Weepy facial expression"
    • Heliotropic erythema of the eyelids
    • Cheeks: butterfly-shaped erythema
    • Shawl sign: perioral recess erythema
    • Atrophic, hypo- or hyperpigmentation
    • Gottron's sign
      • In 70% of patients
      • pathognomic
      • Striated erythema and papules on the finger extensor sides
    • Nailfold hyperkeratoses with telangiectasia and telangiectasia
      • Keining's sign = pain when trying to push back the nail fold
    • Rhagades of the fingertips
    • Scleroderma-like indurations
    • Raynaud's syndrome
    • Uncharacteristic psoriasiform, lichen planus-like exanthema
    • Diffuse effluvium
    • Poikiloderma
  • Musculature:
    • Muscle weakness, especially proximal limb sections (neck, shoulder girdle, legs)
    • Symmetric muscle weakness/ myalgias/ muscle atrophies/ contractures
    • Pharyngeal and laryngeal muscles may also be affected
    • Calcifications of the musculature are possible
    • Dermatomyositis sine myositis or amyopathic dermatomyositis are called dermatomyositides without muscle involvement
  • Bones and joints:
    • Osteoporosis
    • Arthralgias
    • Arthritides
    • Symmetric polyarthritis, also known as oligo- or monarthritis
  • Overlap syndromes are possible
  • Other (rare) organ involvement of
    • Heart
    • Intestine
    • Kidneys
  • Lab
    • All laboratory values listed may be normal despite the presence of dermatomyositis.
    • BSG ↑, CRP ↑.
    • Blood count: leukocytosis, lymphopenia, eosinophilia.
    • ↑ Serum creatinine phosphokinase, aldolase, CPK, GOT, GPT, LDH
    • Antinuclear antibody (ANA) positive in approx. 1/3 of cases.
    • Jo-1 in 20%
    • PM-Scl 5%
    • nRNP 5%
    • Anti-Mi 10-30%
    • PL-7 < 3%
    • PL-12 < 3%
    • OJ in < 3%
    • EJ in < 3%
    • Serum electrophoresis: α2- and γ-globulin.
    • Urine: creatinine and creatinineuria. Proteinuria in relapse, myoglobinuria.

  • Clinic
  • Laboratory:
    • blood sedimentation rate, C-reactive protein, liver and kidney values, creatinine kinase, aldolase, LDH, rheumatoid factor
    • Thyroid antibodies
    • ANAs and ENAs
  • U status/creatin excretion in 24-hour urine
  • Biopsy:
    • Skin biopsy
    • Muscle biopsy
  • Electromyogram: polyphasic potentials
  • Capillaroscopy
  • Tumour exclusion
  • To diagnose "polymyositis", at least 3 of the following criteria should be positive:
    • Muscle enzymes (creatinine kinase)
    • Electromyogram: polyphasic potentials
    • Muscle biopsy

  • Unpredictable
  • 5-year survival rate at 95% or the 10-year survival rate at 84%

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