title: Successful plasma exchange combined with rituximab therapy in aggressive APS-related cutaneous necrosis.
abstract: Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by venous and/or arterial thrombosis or recurrent fetal loss associated with the presence of antiphospholipid antibodies and/or a lupus anticoagulant. The skin appears to be an important target organ and many cases of APS may present with skin manifestations. These lesions may be manifold and may take the form of livedo reticularis, livedo racemosa, ulcerations, digital gangrene, subungeal splinter hemorrhages, superficial venous thrombosis, thrombocytopenic purpura, pseudovasculitic manifestations, extensive cutaneous necrosis, or primary anetoderma. We report a case of fulminant APS-related cutaneous necrosis. A 38-year-old Caucasian male with a past history of APS, multiple deep vein thrombi/pulmonary emboli, presented with necrotic lesions on his right upper and right lower extremities. Initially, baseline anticoagulation was increased without improvement. Subsequently, plasma exchange was initiated on a daily schedule. Furthermore, rituximab 1,000 mg IV was administered on days 1 and 15. After six consecutive daily sessions of plasma exchange, there was significant regression of the necrotic lesions. After a 22-day hospital stay, the patient was discharged to home on fondaparinux. The patient presented approximately 2 months later after missing follow-up appointments. At the time, his initial lesions looked remarkably improved.
authors.affiliation: Division of Hematology/Oncology, Western Pennsylvania Hospital, Western Pennsylvania Cancer Institute, 4800 Friendship Avenue, Suite 2303 NT, Pittsburgh, PA 15224, USA.
authors.forename: Rubens
authors.initials: R
authors.lastname: Costa
authors.forename: Salman
authors.initials: S
authors.lastname: Fazal
authors.forename: Robert B
authors.initials: RB
authors.lastname: Kaplan
authors.forename: Joel
authors.initials: J
authors.lastname: Spero
authors.forename: Ricardo
authors.initials: R
authors.lastname: Costa
mesh_terms: D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000473:pathology / Q000628:therapy*; D000077425:Fondaparinux; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D009336:Necrosis; D010951:Plasma Exchange; D011134:Polysaccharides / Q000627:therapeutic use; D000069283:Rituximab; D012867:Skin / Q000473:pathology*; D017445:Skin Diseases, Vascular / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000925:Anticoagulants; D007155:Immunologic Factors; D011134:Polysaccharides; D000069283:Rituximab; D000077425:Fondaparinux