Thalidomide Effects in Behçet’s Syndrome and Pustular Vasculitis

Joseph L. Jorizzo, Frank C. Schmalstieg, Alvin R. Solomon, Tito Cavallo, Robert S. Taylor, Helen B. Rudloff, Elisabeth J. Schmalstieg, Jerry C. Daniels

Research output: Contribution to journalArticle

80 Scopus citations

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 labeling 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.

Original languageEnglish (US)
Pages (from-to)878-881
Number of pages4
JournalArchives of Internal Medicine
Volume146
Issue number5
DOIs
StatePublished - May 1986

ASJC Scopus subject areas

  • Internal Medicine

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    Jorizzo, J. L., Schmalstieg, F. C., Solomon, A. R., Cavallo, T., Taylor, R. S., Rudloff, H. B., Schmalstieg, E. J., & Daniels, J. C. (1986). Thalidomide Effects in Behçet’s Syndrome and Pustular Vasculitis. Archives of Internal Medicine, 146(5), 878-881. https://doi.org/10.1001/archinte.1986.00360170074012