Comment on: Eculizumab as a rescue therapy in refractory anti-NXP2 dermatomyositis with digestive vasculitis and gastrointestinal perforation: report of two cases

Dear Editor, We read with great interest the article by Combemale et al. [1] that was recently published in Rheumatology. Through the report of two cases, the authors proposed that eculizumab may serve as an effective rescue therapy in digestive vasculitis associated with refractory dermatomyositis (DM) with anti-nuclear matrix protein (anti-NXP2) antibodies (DM-NXP2+). Given the devastating outcomes of gastrointestinal (GI) involvement in DM-NXP2+ [2], this therapeutic success represents a promising strategy to alleviate the mortality of this rare and extremely severe form of the disease. In light of these two case-reports, we would like to highlight several points that captured our attention, particularly regarding the interferon (IFN) and complement pathways, their interactions and the similarities with malignant atrophic papulosis (MAP), referred to as systemic Degos disease. MAP is a rare, often fatal vaso-occlusive disorder, defined by pathognomonic skin lesions and systemic involvement, sometimes occurring in the setting of autoimmune diseases. Fig. 1 illustrates the typical GI involvement of MAP.