Clinically amyopathic dermatomyositis is being advocated as a distinct subcategory of dermatomyositis. When a patient exhibits the characteristic dermatomyositis rash without muscle symptoms, the following criteria should be considered:

  • Skin manifestations typical of dermatomyositis: heliotrope rash, periorbital swelling, Grotton’s papules/sign, V-sign, shawl sign, holster sign
  • Skin biopsy demonstrating reduced capillary density, deposits of membrane attack complex on small blood vessels along the dermal-epidermal junction, and within the walls of dermal blood vessels
  • No objective weakness
  • Normal serum creatine kinase (CK) levels
  • Normal EMG findings
  • Muscle biopsy, if done, does not reveal features compatible with definite or probable DM