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1.
JCEM Case Rep ; 2(4): luae054, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38601066

ABSTRACT

Severe hypertriglyceridemia can be manifested by xanthomas. Therapeutic plasma exchange (TPE) is an invasive medical procedure that has been documented as a viable approach for severe hypertriglyceridemia when cases would be refractory to conventional therapies. TPE is mainly an optional therapeutic modality for cases of severe acute pancreatitis or preventing the recurrence of pancreatitis. Beyond this clinical application, data are scarce on TPE utilization in managing cutaneous lesions associated with hypertriglyceridemia. We present a case of severe hypertriglyceridemia accompanied by extensive xanthomas of various types and a history of recurrent pancreatitis. After conventional therapy failed, a modified plasmapheresis regimen was used and was able to achieve a fast and marked reduction in the patient's serum triglyceride levels with complete resolution of the extensive cutaneous lesions, providing him a newfound comfort he had not experienced in some time and suggesting the regimen potentially could be considered in the treatment of refractory severe hypertriglyceridemia with debilitating cutaneous complications.

2.
J Inflamm Res ; 16: 1431-1441, 2023.
Article in English | MEDLINE | ID: mdl-37034473

ABSTRACT

Background: Spontaneous pneumomediastinum and subcutaneous emphysema are rare and serious complications of dermatomyositis (DM). Case Presentation: Our article presents two clinically heterogeneous cases of DM who developed pneumomediastinum and subcutaneous emphysema. The first was a 24-year-old lady with a recently diagnosed DM. She developed rapidly progressive pneumonia, interstitial lung disease (ILD), pneumomediastinum, subcutaneous emphysema, and acute respiratory distress syndrome (ARDS). She died despite treatment with steroids and immunosuppressants (methotrexate and mycophenolate mofetil (MMF)). The second was a 30-year-old man diagnosed with amyopathic DM. He developed pneumomediastinum prior to ILD, which worsened over time, and subcutaneous emphysema evolved. However, he recovered completely after corticosteroid, MMF, and rituximab. Conclusion: Spontaneous pneumomediastinum and subcutaneous emphysema may complicate DM. Corticosteroids, immunosuppressants, and respiratory support are the mainstay of management for these conditions. Though they were reported to carry a poor prognostic value, the course and outcome are highly variable among the cases.

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