M33

Dermatopolymyositis

A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. from Adams et al., Principles of Neurology, 6th ed, pp1405-6)

Progressive condition characterized by symmetric proximal muscular weakness with elevated serum levels of muscle enzymes and a skin rash, typically a purplish-red erythema on the face, and edema of the eyelids and periorbital tissue; affected muscle tissue shows degeneration of fibers with a chronic inflammatory reaction; occurs in children and adults, and in the latter may be associated with visceral cancer or other disorders of connective tissue.

Juvenile dermatomyositis

Juvenile dermatomyositis organ involvement unspecified

Juvenile dermatomyositis with respiratory involvement

Juvenile dermatomyositis with myopathy

Juvenile dermatomyositis without myopathy

Juvenile dermatomyositis with other organ involvement