Skin Window Examination According to Rebuck and Cutaneous Pathergy Tests in Patients with Behçet’s Disease

Abstract

Comparative in vivo examinations of skin (cutaneous pathergy test, skin window technique over 24 h according to Rebuck) were carried out in 12 patients with Behçet’s disease (BD) and in 13 healthy volunteers serving as controls. 10 out of 12 patients at active phases of BD showed a positive papular (-pustular) skin reactivity to needle prick or vene puncture. These 10 patients simultaneously revealed an increased chemotactic activity of polymorphonuclear leukocytes (PMNL) in vitro. In the controls all pathergy tests were negative and chemotaxis of PMNL was within normal range. The proportion of PMNL initially coming up to 100% in the skin window preparations, very rapidly decreased in patients with BD as compared to a distinctly slower drop in the controls. Conversely to the number of PMNL, the skin window cellular pattern was built up by monophagocytic cells replacing the PMNL. The phenomenon of ‘rapid depletion of PMNL’, from skin window can be considered an additional hint on the functional disturbance of the PMNL involved in the pathogenesis of BD.

Dapsone in Behçet's Disease: A Double‐Blind, Placebo‐Controlled, Cross‐Over Study

“…The non‐specific cutaneous hyperactivity response (pathergy) may serve as an important diagnostic indicator in BD (9). This skin phenomenon is positively correlated with an increase in chemotactic activity of polymorphonucleocytes (PMNs) in patients with BD (12). Sharquie (7) reported the suppressive effects of dapsone on the pathergy test.…”

Advances in pathogenesis and treatment of ocular involvement in Behcet’s disease

“…After this initial genetic discovery, HLA-B51 was then found to have a strong association with BD in 1982 (10). Afterwards, this association was further confirmed in multiple studies among different populations of BD patients (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). HLAB51 allele accounts for about 19% of the genetic susceptibility and is by far the most powerful genetic factor in BD (23,24).…”

Oro-genital ulcers with a positive pathergy test in acute myeloid leukaemia

“…With the presence of oral and genital ulcers, erythema nodosum and a positive pathergy test our patient fulfilled criteria for the diagnosis of Behcet's disease. However, a positive pathergy test rarely occurs in malignant disorders such as hairy cell leukaemia, T cell non-Hodgkin's lymphoma and chronic myeloid leukaemia treated with interferon alpha [4,5,6]. No previous reports suggest an association of a positive pathergy test with acute myeloid leukaemia.…”