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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e3181b83618
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Ischemic Ulcerative Colitis in Juvenile Dermatomyositis

Meneghel, Alessandra MD*; Zulian, Francesco MD; Martini, Giorgia MD; Guariso, Graziella MD*

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*Department of Pediatrics, Gastroenterology-Endoscopy, Italy

Rheumatology Units, University of Padua, Padua, Italy

Received 11 November, 2008

Accepted 17 July, 2009

Address correspondence and reprint requests to Prof. Graziella Guariso, Dipartimento di Pediatria, Via Giustiniani 3, 35128 Padua, Italy (e-mail: guariso@pediatria.unipd.it).

The authors report no conflicts of interest.

A 6-year-old girl with juvenile dermatomyositis (JDM), diagnosed according to the Bohan and Peter criteria (1), presented with skin ulcerations (Fig. 1) and livedo reticularis (Fig. 2). Three months after diagnosis she developed severe iron-deficiency anemia, abdominal pain, evidence of occult blood in the stool, and occasional hematochezia. Colonoscopy showed a serpiginous vascular pattern and 2 ulcers (Figs. 3 and 4). Histological findings on mucosal biopsies showed granulation tissue and mixed inflammatory infiltrate, mostly composed of granulocytes and scattered lymphocytes, with peripheral mucosal hyperplastic-reactive changes (Fig. 5).

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Fig. 3
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Fig. 4
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Fig. 5
Fig. 5
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Among the possible complications of JDM (2–9), gastrointestinal (GI) involvement is rare (3) and ulcers are more typical of the juvenile- than of the adult-onset form (2). GI vasculopathy can manifest as ulceration, haemorrhage, pneumatosis intestinalis, or perforation (4,10). These symptoms and signs may arise early in the course of the disease as a result of a noninflammatory endarteropathy or late because of chronic vasculopathy (2,10).

Vasculitides involving the GI tract are part of a systemic process, although the signs and symptoms may initially be vague (9). GI involvement should be considered in every patient with JDM associated with skin ulcerations and abdominal symptoms, to ensure appropriate immunosuppressive treatment and avoid severe complications.

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REFERENCES

1. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975; 292:344–347.

2. Mamyrova G, Kleiner DE, James-Newton L, et al. Late-onset gastrointestinal pain in juvenile dermatomyositis as a manifestation of ischemic ulceration from chronic endarteropathy. Arthritis Rheum 2007; 57:881–884.

3. Cassidy JT, Lindsley CB. Juvenile dermatomyositis. In: Cassidy, Petty, Laxer and Lindslay, eds. Textbook of Pediatric Rheumatology. Philadelphia: Elsevier-Saunders; 2005. pp. 407–41.

4. Wang IJ, Hsu WM, Shun CT, et al. Juvenile dermatomyositis complicated with vasculitis and duodenal perforation. J Formos Med Assoc 2001; 100:844–846.

5. Prahalad S, Bohnsack JF, Maloney CG, et al. Fatal acute fibrinous and organizing pneumonia in a child with juvenile dermatomyositis. J Pediatr 2005; 146:289–292.

6. Rider LG, Atkinson JC. Images in clinical medicine. Gingival and periungual vasculopathy of juvenile dermatomyositis. N Engl J Med 2009; 360:e21.

7. Yun SJ, Lee JB, Kim SJ, et al. Calcinosis cutis universalis with joint contractures complicating juvenile dermatomyositis. Dermatology 2006; 212:401–403.

8. Jat KR, Singh S. Calcinosis in juvenile dermatomyositis. Indian Pediatr 2008; 45:784.

9. Cucchiara S, Borrelli O. Vasculitides. In: Guandalini S, editor. Textbook of Pediatric Gastroenterology and Nutrition. London: Taylor & Francis Group; 2004. pp. 419–434.

10. Feldman BM, Rider LG, Reed AM, et al. Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. Lancet 2008; 371:2201–2212.

© 2009 Lippincott Williams & Wilkins, Inc.

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