Juvenile amyopathic dermatomyositis.
We report a 15-year-old girl with a 10-year-old history of typical skin features of dermatomyositis (DM) without evidence of muscle involvement. Amyopathic dermatomyositis (ADM) is defined by the presence of biopsy confirmed classic cutaneous findings of dermatomyositis in the absence of any clinical or laboratory signs of muscle disease for at least 2 years after onset of skin pathology. To exclude muscle involvement muscle enzymes should be normal; moreover additional use of magnetic resonance imaging and muscle ultrasound is currently being proposed. It is as yet undetermined, whether early aggressive immunosuppressive treatment of ADM might prevent the development of myositis at a later date or influence the course of the skin disease. In a paediatric patient with ADM we advocate a more expectant attitude with careful and regular monitoring for possible development of muscle disease.
Schmid
M H
MH
Department of Dermatology, University Hospital of Zürich, Switzerland.
Trüeb
R M
RM
D000293:Adolescent; D003882:Dermatomyositis / Q000473:pathology*; D005260:Female; D006801:Humans; D012867:Skin / Q000473:pathology*