title: Prothrombotic state in Churg-Strauss syndrome: a case report.
abstract: An increased risk of thrombosis has been described in patients with hypereosinophilic syndromes, including Churg-Strauss syndrome (CSS). We report the case of a 43-year-old man with CSS who presented with asthma, pansinusitis, blood eosinophilia (9650/microL), peripheral neuropathy, cutaneous eosinophilic vasculitis, and a positive result for antineutrophil cytoplasmic antibodies. An analysis of plasma during active disease revealed elevated levels of prothrombin fragment 1+2 (marker of thrombin generation) (832 pM; normal range, 68-229 pM) and D-dimer (marker of fibrin degradation) (2300 ng/mL; normal range, 130-250 ng/mL), which indicate an increased risk of thrombosis. Both parameters returned to normal values during remission after immunosuppressive treatment. Skin histology showed leukocytoclastic vasculitis with numerous eosinophils in the dermal infiltrate. Immunohistochemistry revealed expression of tissue factor by skin-infiltrating eosinophils, as confirmed by colocalization with eosinophil cationic protein, a classic marker of eosinophil granulocytes. In conclusion, we present a patient with active CSS and a prothrombotic state that reverted during remission achieved by immunosuppressive therapy.
authors.affiliation: U.O. Dermatologia, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: A V
authors.initials: AV
authors.lastname: Marzano
authors.forename: A
authors.initials: A
authors.lastname: Tedeschi
authors.forename: R
authors.initials: R
authors.lastname: Rossio
authors.forename: D
authors.initials: D
authors.lastname: Fanoni
authors.forename: M
authors.initials: M
authors.lastname: Cugno
mesh_terms: D000328:Adult; D015267:Churg-Strauss Syndrome / Q000097:blood* / Q000188:drug therapy; D004802:Eosinophilia / Q000188:drug therapy / Q000209:etiology; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D010446:Peptide Fragments / Q000097:blood; D011516:Prothrombin; D012871:Skin Diseases / Q000188:drug therapy / Q000209:etiology; D013925:Thromboplastin / Q000032:analysis / Q000276:immunology; D013927:Thrombosis / Q000209:etiology* / Q000276:immunology
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; D007166:Immunosuppressive Agents; D010446:Peptide Fragments; C036309:fibrin fragment D; C045879:prothrombin fragment 1.2; D011516:Prothrombin; D013925:Thromboplastin