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Juvenile Dermatomyositis

An autoimmune disease with skin and muscle involvement in childhood.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Juvenile Dermatomyositis?

Juvenile dermatomyositis (JDM) is an autoimmune inflammatory disease of the skin and proximal muscles starting under 18 years of age. Its incidence is 2-4/1,000,000. It shows bimodal age distribution (5-9 years and 10-14 years).

Pathogenesis: it is autoimmune vasculopathy progressing with microangiopathy and perifascicular atrophy. Genetic (HLA-DRB1) and environmental triggers (viral, UV) play a role.

Treatment is corticosteroids, methotrexate and intravenous immunoglobulin. Biological agents are used in resistant cases. Calcinosis cutis, chronic muscle atrophy and lung involvement are serious complications.

Symptoms

Heliotrope rash (eyelid, purple-red)
Gottron papules (MCP and IFP joints)
Proximal muscle weakness
Difficulty climbing stairs, combing
Dysphagia (esophageal involvement)
Calcinosis cutis (advanced cases)

Risk Factors

Female gender (2:1)
Genetic predisposition (HLA-DRB1)
History of viral infection
UV light exposure
Family history of autoimmune disease
Age 5-9 or 10-14 years

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Characteristic purple rash on face
  • Difficulty climbing stairs or fatigue
  • Rashes over hand joints
  • Muscle pain and weakness
  • Dysphagia and voice change

Treatment Methods

01
High-dose corticosteroid (prednisolone)
02
Methotrexate (steroid sparing)
03
Intravenous immunoglobulin (IVIG)
04
Rituximab, cyclophosphamide (resistant)
05
Physiotherapy and rehabilitation
06
Sun protection and skin care

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.