Juvenile dermatomyositis resembling late-stage Degos disease with gastrointestinal perforations successfully treated with combination of cyclophosphamide and rituximab: case-based review.

Dermatomyositis (DM) is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle. Small blood vessel injury in the GI tract has been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation. Recent histopathological studies have shown deposits in the capillaries of the skin, gastrointestinal tract, and brain of patients with dermatomyositis similar to that found in patients with Degos disease, suggesting these disease processes are closely related or represent varying degrees of severity on the same pathologic spectrum.

Evaluation of Rapid vs Standard Infliximab Infusions in the Pediatric Population.

Background: Rapid 1-hour infliximab infusions have been safely implemented in adults, but studies of these rapid infusions in pediatric patients are limited. This study's primary objective was to determine the safety of 1-hour infliximab infusions compared with standard 2- to 3-hour infusions in children with inflammatory bowel disease and other autoimmune disorders.

Methods: We conducted an institutional review board-approved prospective study using an unmatched historical control group at a freestanding children's hospital comparing rapid vs standard infusion rates of infliximab and the use of premedications and immunomodulatory agents on the frequency of early and delayed infusion reactions.