Malignant Atrophic Papulosis

Abstract

We thank the patient for permission to publish this information. We also thank Elisabeth Homassel for her technical assistance.

Degos disease: a case report and review of the literature

“…The differential diagnosis includes primary antiphospholipid syndrome or the antiphospholipid syndrome caused by systemic lupus erythematous or other connective tissue diseases. Malignant atrophic papulosis is a vaso-occlusive disorder of unknown cause [ 9 , 12 ].…”

“…Immunosuppression with corticosteroids has been shown to worsen skin lesions and further complicate the course of the disease [ 5 ]. The efficacy of eculizumab (monoclonal antibody targeted against complement C5) and treprostinil (synthetic prostaglandin agonist) has been reported in the literature [ 9 , 12 ]. For gastrointestinal tract perforation, surgical intervention is the only choice however recurrent perforations may still develop [ 9 , 12 ].…”

“…The efficacy of eculizumab (monoclonal antibody targeted against complement C5) and treprostinil (synthetic prostaglandin agonist) has been reported in the literature [ 9 , 12 ]. For gastrointestinal tract perforation, surgical intervention is the only choice however recurrent perforations may still develop [ 9 , 12 ]. Systemic manifestations might develop suddenly or even years after the occurrence of skin lesions, indicating the need for annual follow-up [ 11 ].…”

Asymptomatic skin lesions with bowel perforation and cerebrovascular stroke in a young adult male

“…It has unclear etiology; viral infection, autoimmune disease, coagulopathy, and collagen vascular disorders have all been suggested as underlying pathophysiologies 2 . It also can be manifesting with other connective tissue disorders such as systemic lupus erythematosus (SLE), dermatomyositis, or antiphospholipid antibody syndrome 3 …”

“…Treatment modalities for Degos disease usually include aspirin, Dipyridamole, heparin, pentoxifylline, steroids, cyclophosphamide, and nicotinamide, but they have limited success 1 . Eculizumab (A C5b‐9/Interferon‐α inhibitor) and treprostinil are new treatment options used recently with promising results 3 …”