Case reportA fatal presentation of dermatomyositis with facial swelling
Section snippets
Case report
A 13-year-old African American male presented with a two-week history of bilateral periorbital swelling, decreased appetite and fatigue. Because of a history of both eczema and allergies to pollens, aspirin, and peanuts, he was treated with anti-histamines and topical steroids. When the swelling did not improve, a CT scan of his sinuses was performed that showed inflammation lining the maxillary, ethmoid and sphenoid sinuses. He was treated with azithromycin (Z-Pak) and Omnicef. Although there
Final diagnosis
The biopsy of the sternocleidomastoid muscle showed the presence of inflammatory cells along with perifascicular necrosis suggesting severe active necrotizing myopathy, consistent with juvenile dermatomyositis (JDM) (Fig. 1, panel III).
Hospital course
Later that evening, the patient developed bloody diarrhea, which led rapidly to metabolic acidosis, hyperkalemia, and ventricular fibrillation. Despite resuscitative measures and replacement fluids, he succumbed the following day. A final autopsy revealed two large ulcers in the duodenum with erosion into a submucosal blood vessel. A massive amount of blood was found in the peritoneal cavity indicating intestinal perforation as a cause of hemorrhage and death. Examination of the skeletal
Discussion
Our patient first came to medical attention with the onset of periorbital edema. He had normal muscle strength and did not display the typical juvenile dermatomyositis skin rash (Gottron's papule, heliotrophic rash or Shawl sign). Although childhood dermatomyositis classically presents with insidious progressive muscle weakness, others have reported cases of JDM presenting as periorbital edema [1]. A retrospective analysis performed on the charts of 50 children at Children's Hospital, Los
Conclusion
Here we describe a case of juvenile dermatomyositis (JDM) with an unusual presentation consisting of bilateral periorbital edema, increased muscle enzymes, and rapid death within three months of the initial presentation due to intestinal hemorrhage. In one large-scale study of JDM it was found that the average evolution time for the disease before consultation is about 8.5 months [6]. This case highlights the need to maintain high vigilance in diagnosing and treating JDM, especially in cases in
Funding
None.
Conflict of interest
All authors have no conflict of interest to declare.
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2012, Current Opinion in OphthalmologyA fatal presentation of dermatomyositis
2012, Kathmandu University Medical Journal