0 avis
Rescue Therapy in Malignant Atrophic Papulosis: A New Era of Promising Treatments Targeting Interferon and Complement Pathways.
Archive ouverte
Edité par CCSD -
International audience. Dear Editor, Malignant atrophic papulosis (MAP), also known as systemic Degos disease, is a rare and often fatal vaso-occlusive disorder characterized by ischaemic skin lesions and potential multiorgan involvement, leading to significant morbidity and mortality. The hallmark of AP includes papules with a porcelain-white atrophic centre and erythematous border, with histopathological findings of wedge-shaped necrosis and small vessel occlusion.1,2 Skin-limited Degos disease has a relatively benign course (benign AP or BAP); however, systemic involvement substantially worsens the prognosis, with mortality rates reaching up to 76% in patients with severe organ involvement, such as intestinal perforations and cerebral infarctions.1,3,4 Pathogenesis is characterized by the upregulation of type-I interferons and deposition of the C5b–9 complex (membrane attack complex) on vessels, which leads to obliterative vasculopathy.1,2,5,6 A combination of eculizumab and treprostinil has been reported to be promising in preventing gastrointestinal MAP relapses; however, data remain scarce, and progressive neurologic, pleural and pericardial MAP has been reported in patients receiving these treatments.1,4,5 We present a patient with MAP successfully treated with a combined therapy targeting both type-I interferon and complement pathways.
Consulter en ligne
Suggestions
Du même auteur
