Clinical Case Vignettes: Connective Tissue Diseases in Children

Fig. 32.1

Calcinotic nodules in a child with juvenile dermatomyositis

At the end of 6-year follow-up, her muscle weakness was in remission for >2 years, and methotrexate could be completely stopped. At 7-year follow-up, patient was not on any drugs and was in remission with major reduction in calcinosis.

Calcinosis is a common manifestation of JDM seen in almost 40 % patients and can cause significant morbidity [9]. It is a dystrophic calcification which can affect skin, muscle, or fascia. It is usually seen in patents that have had persistent disease activity or are treated late. There are no approved treatment guidelines for it; however, treatment with bisphosphonates has been shown to be beneficial in several case series and reports [10, 11]. Commonly used agents are alendronate and pamidronate.

Take-Home Messages

1.

In children with JDM, calcinosis can occur even when the muscle disease is in remission.
2.

Bisphosphonates may be useful in reducing the calcinosis.

Conclusion

Multisystem involvement should suggest a diagnosis of connective tissue disease after common diseases have been ruled out. Mimics should also be ruled out such as scleredema for systemic sclerosis, dermatomyositis for SLE, and infection and malignancy for SLE.

References

Biner B, Acunaş B, Karasalihoğlu S, Vatansever U. Systemic lupus erythematosus presenting with generalized lymphadenopathy: a case report. Turk J Pediatr. 2001;43:94–6.PubMed

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