juvenile dermatomyositis
Pronunciation: der-ma-toe-my-oh-SY-tis
Also written as: JDM — juvenile dermatomyositis
A rare inflammatory muscle disease in children characterized by proximal weakness and distinctive skin rashes.
Full Definition
Juvenile dermatomyositis (JDM) is a rare, chronic, multisystem autoimmune disease affecting approximately 3-5 children per million annually. The condition is characterized by proximal muscle weakness, particularly affecting the shoulders and hips, combined with pathognomonic skin changes including heliotrope rash around the eyes and Gottron's papules over the knuckles. Unlike the adult form, JDM rarely associates with malignancy but can involve significant vasculopathy affecting multiple organ systems. Early aggressive treatment with corticosteroids and immunosuppressive agents is crucial to prevent permanent muscle damage and calcinosis.
Usage
Usage note: Always distinguish from adult dermatomyositis due to different clinical features and prognosis.
In Context
- "The characteristic heliotrope rash and proximal weakness confirmed the diagnosis of juvenile dermatomyositis." — Diagnostic workup
- "Early treatment of juvenile dermatomyositis prevented the development of calcinosis in this patient." — Long-term follow-up