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Dermatomyositis and Whipple's disease.

T R Helliwell1, R E Appleton, N C Mapstone

  • 1Department of Pathology, University of Liverpool, UK.

Neuromuscular Disorders : NMD
|March 11, 2000
PubMed
Summary

This case study reveals a rare association between juvenile dermatomyositis and Whipple's disease in a 14-year-old boy. Early diagnosis and combined treatment with antibiotics and immunosuppressants are crucial for managing this complex condition.

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Area of Science:

  • Pediatrics
  • Rheumatology
  • Infectious Diseases

Background:

  • Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy characterized by skin rash and proximal muscle weakness.
  • Whipple's disease is a rare systemic bacterial infection caused by Tropheryma whippelii, typically affecting the joints, heart, and central nervous system.

Observation:

  • A 14-year-old male presented with JDM symptoms including rash, proximal weakness, myalgia, and weight loss.
  • Muscle biopsy revealed characteristic JDM findings and ultrastructural evidence of debris.
  • Following initial treatment for JDM, the patient developed cardiac complications and rash recurrence.

Findings:

  • Polymerase chain reaction (PCR) analysis detected Tropheryma whippelii DNA in skeletal and cardiac muscle.

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  • The patient's symptoms improved with a combination of prednisolone and co-trimoxazole, alongside verapamil for tachycardia.

    • Implications:

    • This case suggests a potential novel association between JDM and Whipple's disease.
    • The findings highlight the importance of considering infectious etiologies in refractory or recurrent JDM cases.
    • Combined immunosuppressive and antibiotic therapy may be effective in managing this rare dual diagnosis.