Background: The collagen vascular diseases are a collection of conditions, which are thought to be secondary to pathologic alterations in the immune system. Deposition of immune complexes in blood vessel walls resulting in either ischemia or thrombosis is the most widely accepted pathologic mechanism. The lack of familiarity with this subgroup of disease can lead to unnecessary surgical intervention.
Data sources: A Medline search was performed of all the English-language literature. Further references were obtained through cross-referencing the bibliography cited in each work.
Conclusion: Clinical manifestations are varied and complications include constipation, fecal incontinence, pseudoobstruction, perforation, hemorrhage, and mesenteric ischemia. Colorectal manifestations typically follow dermal presentations. Management should be conservative especially for pseudo-obstructions. Surgical intervention increases morbidity and should be chosen when absolutely necessary. Because of the high incidence of colorectal malignancies in patients with dermatomyositis, aggressive screening should be performed.