Introduction:

Dermatomyositis belongs to the heterogeneous group of autoimmune inflammatory myopathies, whereas Madelung disease is a rare metabolic disorder characterized by symmetrical, non-encapsulated adipose tissue deposits.

Objective:

To the best of our knowledge, we present the first case in the literature with the coincidence of these two diseases.

Case report:

The patient was diagnosed with juvenile dermatomyositis at the age of 7 and was treated with steroids, azathioprine, and intravenous immunoglobulins. The diagnosis of Madelung disease was based on the physical examination and computed tomography scans when she was 42 years old. The patient declined surgical treatment for Madelung disease, and fibrate therapy did not provide any relief from her symptoms associated with the disease.

Conclusions:

We present the case of the coexistence of juvenile dermatomyositis, requiring long-term steroid therapy, with Madelung disease. Further research is needed to assess whether a diagnosis of juvenile dermatomyositis may increase the risk of developing Madelung disease.