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Lower extremity lipedema, upper extremity lipodystrophy and severe calcinosis complicating juvenile dermatomyositis
Slavica R Pavlov-Dolijanovic1, Nada Z Vujasinovic Stupar, Nikola Gavrilov
1Institute of Rheumatology Belgrade, Resavska 69, Belgrade, Serbia, dolijan@eunet.rs.
Insights
Juvenile dermatomyositis (JDM) can cause significant complications, including lipodystrophy and lipedema. This case highlights a rare presentation of JDM with co-occurring lipedema and lipodystrophy, raising questions about autoimmune disease overlap.
Area of Science:
- Rheumatology
- Pediatric Autoimmunology
- Dermatology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease in children, often leading to chronic complications like muscle atrophy, contractures, and calcinosis.
- Recent reports suggest potential body fat redistribution in JDM patients, with the nature of this lipodystrophy, particularly its relation to lower extremity lipedema, remaining unclear.
Observation:
- A 39-year-old woman, diagnosed with JDM at age 7, presented with symmetrical upper extremity lipodystrophy and lower extremity lipedema.
- She also had progressive, subcutaneous calcified nodules and scleroderma-like capillaroscopy findings.
- Positive ANA and anti-U1RNP antibodies were noted during her follow-up.
Findings:
- This case represents a unique simultaneous occurrence of lower extremity lipedema, upper extremity lipodystrophy, and severe calcinosis in a JDM patient.
- Calcinosis and lipodystrophy were found to be associated with a shorter duration of active JDM disease.
- The patient's presentation suggests a potential for overlapping autoimmune conditions during the long-term follow-up of JDM.
Implications:
- This case expands the known clinical spectrum of JDM complications, particularly regarding body fat redistribution and its association with lipedema.
- It underscores the importance of recognizing and investigating complex presentations in JDM, including potential co-existing autoimmune disorders.
- Further research is warranted to understand the pathogenesis and management of these rare JDM-associated conditions.
Abstract:
Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood. Significant proportions of patients have residual weakness, muscle atrophy, joint contractures, and calcinosis. Recently, new clinical findings, such as lipodystrophy accompanied with increased fat deposition in certain areas, have been reported. So far, it is not known whether the redistribution of body fat may be the type of lipedema of lower extremity. We describe a 39-year-old woman who was diagnosed with JDM at the age of 7. Later she developed symmetrical lipodystrophy of upper extremities and symmetrical lipedema of lower extremities (making 2 and 58.3 % of total body fat mass, respectively), with multiple calcified nodules in the subcutaneous tissues. These nodules gradually increased in size despite therapy. Capillaroscopy findings showed scleroderma-like abnormalities. ANA and anti-U1RNP antibodies were positive. Similar cases with simultaneous occurrence of the lipedema of lower extremities, lipodystrophy of upper extremities, and severe calcinosis complicating JDM have not been published so far. We showed that the calcinosis and lipodystrophy were associated with short duration of active disease. Also, we display case that raises the question whether it is possible overlapping autoimmune diseases revealed during follow-up.
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