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A Controlled Trial of Azathioprine in Behçet's Syndrome

Cytotoxic agents have long been used in Behcets syndrome, especially for eye involvement, but their effectiveness has been uncertain. We conducted a two-year randomized, placebo-controlled, double-blind trial of azathioprine (2.5 mg per kilogram of body weight per day) in Turkish men with Behcets syndrome without eye disease (group 1; n = 25) or with eye disease (group 2; n = 48). Corticosteroid treatment remained available to all the patients. All six patients withdrawn from the study because of severe eye disease were receiving placebo (P less than 0.001). Azathioprine was superior to placebo in the prevention of new eye disease in group 1 (1 vs. 8 patients; P less than 0.01) and in group 2 among the 14 patients who at entry had disease in only one eye (P less than 0.001). There were fewer episodes of hypopyon uveitis (1 vs. 15; P less than 0.001) among the group 2 patients who took azathioprine. The patients taking azathioprine also had less frequent oral ulcers, genital ulcers, and arthritis. There were no serious side effects attributable to azathioprine. We conclude that azathioprine is effective in controlling the progression of Behcets syndrome, especially its most serious manifestation, eye disease.

Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome.

Eye disease, arthritis, folliculitis, and thrombophlebitis were more common among males, and erythema nodosum among females, in 297 patients with Behćet syndrome (BS) at their first visit despite an identical disease duration. Younger males and females (age of onset 24 years or less) had a higher prevalence of eye disease and total clinical activity than did the older patients (age of onset 25 years or more). Among the 51 patients followed up for 52 months (SD 7 months) the total clinical activity became significantly less (p less than 0.05) in the whole group at the end of this period. This was particularly true for older females. While male sex and a younger age of onset are associated with more severe disease in BS, overall, the syndrome ran an improving or stable course over 4 1/2 years.

Low dose cyclosporin A versus pulsed cyclophosphamide in Behcet's syndrome: a single masked trial.

A single masked trial of cyclosporin A 5 mg/kg/day versus monthly 1 g intravenous boluses of cyclophosphamide was conducted among 23 patients with Behçets syndrome and active, potentially reversible uveitis. The trial was unmasked after a mean of 12 (SD 2) months for the cyclosporin A group (n = 12) and a mean of 10 (SD 3) months for the cyclophosphamide group (n = 11). During the initial 6 months the visual acuity significantly improved (p < 0.001) in the cyclosporin A group whereas this was not observed in the cyclophosphamide group. The subsequent follow-up of patients up to 24 months suggested that the initial improvement in visual acuity with cyclosporin A was not sustained. More extensive and especially long-term studies of cyclosporin A in the uveitis of Behçets syndrome are warranted.

The arthritis of Behçet's disease: a prospective study.

A prospective study of arthritis was performed in 47 patients with Behçets disease followed up over a 47-month period (mean 19.25 months, SD 14.09). These patients had a total of 80 episodes of arthritis, which were analysed for joint distribution and symmetry, in 56 of which the duration could also be determined. Attacks were oligoarticular, affecting up to 4 joints per patient, 54 (68%) being monoarticular. Knees, ankles, and wrists were the most commonly involved joints. Involvement of spinal, shoulder, hip, and sacroiliac joints was rare. The arthritis was usually not deforming and subacute; 82% (46/56) of the attacks lasted for 2 months or less and 18% (10/56) for between 3 months and 4 years. The ESR was moderately elevated during the attacks. In 32 specimens the synovial fluid was inflammatory (cell count 14.7 +/- 10.1 x 10(9)/l), but in 19 (59%) a good mucin clot formed. Synovial biopsy in 12 patients revealed superficial ulceration, paucity of plasma cells, and in 5 instances lymphoid follicle formation.

Clinicopathologic Evaluation of Nodular Cutaneous Lesions of Behçet Syndrome

Among the cutaneous manifestations, nodular lesions are rather common in Behçet syndrome. The histologic nature of these lesions has been a matter of controversy. To establish their distinguishing features, biopsy specimens from nodular lesions of 24 patients with Behçet syndrome, 25 with nodular vasculitis (NV), and 20 with erythema nodosum (EN) were compared. Statistical analysis revealed insignificant differences between most of the histologic features of Behçed syndrome and NV. However, neutrophil-predominating infiltrate in the subcutis was more common in Behçet syndrome, while necrosis and granuloma formation were encountered more frequently in NV. The differences between Behçed syndrome and EN were more significant. Septal panniculitis, lymphocyte-predominating infiltrate, absence of many vascular changes as well as vasculitis, and necrosis were features in favor of EN. Nodular lesions of Behçet syndrome are mainly neutrophilic vascular reactions with histologic features similar to NV but significantly differing from EN associated with other systemic diseases.

Behçet's Disease: Relation of Serum C-reactive Protein and Erythrocyte Sedimentation Rates to Disease Activity

ABSTRACT: In a prospective study of 150 patients with Behcets disease, significant associations were shown between CRP positivity and the presence of erythema nodosum (p < 0.02) and acute thrombophlebitis (p < 0.05) and between ESR and erythema nodosum (p < 0.01), acute thrombophlebitis (p < 0.001), and acute arthritis (p < 0.01). Mucocutaneous, ocular, or central nervous system activities did not show significant associations with these indices of inflammation. In 50 patients in whom quantitative CRP determinations were performed, clinical disease activity was accompanied by slight to moderate increases in CRP and ESR.

Short term cyclosporin A treatment of Behcet's disease.

Eleven separate three-month courses of cyclosporin A, an oral solution 10 mg/kg/day, were administered to eight patients with Behçets disease with sight-threatening posterior uveitis. It was found to be effective in arresting the inflammatory activity in the eye as well as the mucocutaneous lesions of Behçets disease. Improvement in visual acuity was observed within one week of starting therapy. Severe exacerbations in the ocular and mucocutaneous lesions occurred on withdrawal of the drug. At this dosage side effects included hirsutism in all women, and a slight rise of serum bilirubins in two patients and of blood urea in one patient. The latter two conditions responded rapidly to dose adjustment.

Comparison of two surgical approaches for treating vitiligo: a preliminary study

Background Surgical techniques have recently been introduced for patients with vitiligo. Randomized controlled trials have not been performed.

The role of Helicobacter pylori infection in skin diseases: Facts and controversies

Helicobacter pylori is a Gram-negative bacterium that is considered a causative agent of peptic ulcer disease, gastric lymphoma, and gastric carcinoma. H pylori triggers an intense leucocyte infiltration of the gastric submucosa, an action that is mediated by proinflammatory cytokines. Because this pathogenetic mechanism is common to many other diseases, H pylori seroprevalence has also been investigated in other diseases. H pylori seropositivity is associated with various dermatologic disorders. Although the precise role of H pylori is unknown in these diseases, the organism can be eradicated, using simple and reliable drug regimens. This contribution highlights the dermatologic diseases associated with H pylori seropositivity.

Soaps, shampoos, and detergents

In clinical dermatology, few questions are asked more commonly than “What is the best way for me to clean my skin or hair?” The modern dermatologist needs to be familiar with the physiologic action of cleansing products available on the market. This familiarity should include chemical composition, application, cosmetic benefits, and safety, as well as potential hazards. This knowledge must be acquired from objective, independent, peer-reviewed scientific literature, and not from promotional sheets or biased, pseudoscientific articles that are distributed in great quantity by the cosmetic companies, which have heavily vested commercial interests. It is our hope that this chapter will be such an objective and evenhanded source of information for guiding practicing dermatologists.