title: Thalidomide effects in Behçet's syndrome and pustular vasculitis.
abstract: Pustular vasculitis is a new disease concept that links cutaneous, and possibly systemic, aspects of Behçet's, bowel bypass, bowel-associated dermatosis-arthritis, and disseminated gonorrhea syndromes. The pathomechanism of pustular vasculitic lesion generation may relate to circulating immune complex (CIC)-mediated vessel damage and serum enhancement of neutrophil migration. Thalidomide, an oral pharmaceutical available on strict protocol, has therapeutic effects based on proposed modulation of CIC- and neutrophil-mediated cytotoxicity. Thalidomide therapy was started for four patients with significant morbidity from Behçet's syndrome and for one patient with bowel-associated dermatosis-arthritis syndrome. Clinical benefit was dramatic in all patients who completed sequential four-week "on" and "off" thalidomide therapeutic cycles. In three of four patients, in vivo testing for CIC after histamine injection immunopathology converted from positive (immunoreactant deposition in dermal vasculature [four hours after histamine] and CIC-mediated vasculitis [24 hours after histamine]) to negative during therapy. No effects were noted on neutrophil migration or on the LFA-1/Mac-1/p150,95 family of glycoproteins associated with neutrophil adherence as assessed qualitatively by tritium labelling of neutrophil cell surfaces. In this small patient group, thalidomide was a clinically effective, safe (with rigid monitoring) therapy whose mechanism of action may relate more to inhibitory effects on CIC-induced vasculitis than to effects on neutrophil-mediated cytotoxicity.
mesh_terms: D000936:Antigen-Antibody Complex / Q000032:analysis; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy* / Q000276:immunology; D002460:Cell Line; D002464:Cell Migration Inhibition; D006023:Glycoproteins / Q000032:analysis; D008562:Membrane Glycoproteins; D009504:Neutrophils / Q000276:immunology; D012867:Skin / Q000276:immunology / Q000473:pathology; D013792:Thalidomide / Q000009:adverse effects / Q000627:therapeutic use*; D014657:Vasculitis / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology
chemical_list: D000936:Antigen-Antibody Complex; D006023:Glycoproteins; D008562:Membrane Glycoproteins; C030340:elastin microfibril interface located protein; D013792:Thalidomide