Abstract
We present the clinical and pathologic features of two patients with juvenile dermatomyositis (DM) with severe gastrointestinal ulceration or perforation who required surgery. Abdominal pain which is persistent, progressive or severe, even in patients with improving or mildly active juvenile DM, should be carefully evaluated. The absence of occult blood in the stool and normal radiographs do not exclude these potentially serious complications. Chronic endarteropathy, not an acute vasculopathy previously reported, is the pathology associated with the ischemic ulceration in these patients. The current approach to treatment of juvenile DM with immunosuppressive agents may have contributed to delay in the onset of these gastrointestinal manifestations and to their pathologic features.
MeSH terms
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Abdominal Pain / etiology*
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Abdominal Pain / pathology
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Adolescent
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Arteries / pathology*
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Chronic Disease
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Combined Modality Therapy
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Dermatomyositis / complications*
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Dermatomyositis / drug therapy
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Dermatomyositis / pathology
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Drug Therapy, Combination
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Female
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Humans
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Immunoglobulins, Intravenous / therapeutic use
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Immunosuppressive Agents / therapeutic use
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Intestinal Perforation / etiology
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Intestinal Perforation / pathology
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Intestinal Perforation / surgery
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Intestines / blood supply
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Intestines / pathology
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Intestines / surgery
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Ischemia / etiology*
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Ischemia / pathology
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Ischemia / surgery
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Peripheral Vascular Diseases / complications*
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Peripheral Vascular Diseases / pathology
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Peripheral Vascular Diseases / surgery
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Prednisone / therapeutic use
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Treatment Outcome
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Ulcer / etiology*
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Ulcer / pathology
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Ulcer / surgery
Substances
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Prednisone