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Objective. To assess the influence of being free of major organ involvement during the early years of the disease on the prognosis of men with Behç et's syndrome (BS). Methods. Ninety-six men with BS, who had only active mucocutaneous manifestations when entering a controlled trial of thalidomide mean (S.D.) 11.7 (0.8) years ago, were re-evaluated for the use of immunosuppressives as an indication of major organ involvement during the post-trial period. Results. Outcome information was obtained in 91 (95%) patients. Thirty-nine (43%) patients had to use immunosuppressives during the post-trial period. Immunosuppressive use was significantly more frequent among patients developing BS at younger age (⩽24 years) than older age (30%; 525 years). Developing BS at young age (OR=6.3; 95% CI 2.09, 19.04) and not using colchicine during the posttrial period (OR=3.860; 95% CI 1.484, 10.034) were risk factors for immunosuppressive use. However, 82% of the patients using colchicine had onset during old age. Colchicine showed a significant effect in decreasing the use of immunosuppressives only among patients of old age at onset (Fisher's exact test=5.026; P=0.031) in the subgroup analysis. Eye disease (18 patients) and vascular involvement (14 patients) were the most frequent indications for immunosuppressive use. Conclusions. Being free of major organ involvement during the early years of BS does not indicate a mild prognosis for men developing BS at young age. Whether colchicine will reduce the need for immunosuppressive use among men developing BS at old age awaits formal studies. [ABSTRACT FROM AUTHOR]

Objectives. There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-α administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study. Methods. Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for ≥ 1 year were studied. When they had at least two OUs with a total diameter of ≥ 4mm, they were randomly allocated to (i) 2000 IFN-α IU/day, (ii) 1000 IFN-α IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12. Results. Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint. Conclusions. Low-dose natural human IFN-α did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies. Trial registration. ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184 [ABSTRACT FROM AUTHOR]

Background Because there is an increased inflammatory response to trauma, particularly of the skin, in patients with Behçet’s syndrome (BS), an alteration in wound healing in BS is expected. The aim of this study was to investigate the healing features of punch biopsy wounds in BS and acne vulgaris (AV) patients used as controls. Method Full-thickness skin punch biopsies (4 mm) were taken from the hairless sites of the non-dominant forearms in 20 BS and 20 AV patients. Each patient was examined on days 1, 2, 3, 4, 5, 8, and 10, and the biopsy wound area and induration were marked on sterile glass slides. Other inflammatory changes, such as suppuration and pain, were also recorded. No antiseptic solutions or ointment, except saline, were used. Results The wound area healed similarly in both groups (p > 0.05). Thirteen (65%) BS patients had erythematous haloes around the wound on the first day. The number of patients presenting inflammatory changes reached 18 (90%) on the second day in the BS group. The area of erythema around the wound in BS patients was significantly greater than that in AV patients (5; 25%). It gradually decreased day by day. Purulent changes were observed in four BS patients on day 1, and induration around the biopsy wound in six patients on day 2. Only one patient with AV had suppuration, which appeared on the second day, while no AV patient displayed induration. Conclusion Biopsy-induced trauma may cause increased inflammation in BS, but wound healing is not altered. [ABSTRACT FROM AUTHOR]

Lichen sclerosus is a chronic, skin disease. The involvement of the vagina is not a characteristic of lichen sclerosus. We report a rare case of vulvovaginal syndrome as a complication of lichen sclerosus with the diagnosis and the management of vaginal synechia leading to intravaginal fluid accumulation mimicking pelvic mass. There were two interesting aspects of this case. First, the location of the synechia was behind the introitus, in the vagina. The second was the presence of a pelvic cyst in the lower pelvis. The surgical correction of the case was accomplished with vulvoperninoplasty but the difficulty of this procedure was the identification of the vaginal mucosa after dissection of the synechia. On the next step, the mucosa was attached to the vulvar skin with separate sutures. The postsurgical corticosteroid treatment and the regular dilatation of the introitus are essential to prevent the restenosis. [ABSTRACT FROM AUTHOR]

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The article presents a case study of a six and a half year old male patient and his five and a half year old sibling who were both diagnosed with Netherton syndrome, a rare autosomal recessive genodermatoses. A discussion of the causes and prognosis that are associated with the syndrome is presented.

Purpose: To evaluate safety and efficacy of photodynamic therapy with verteporfin for subfoveal choroidal neovascularization associated with angioid streaks.Design: Prospective interventional case series.Methods: Eight patients (eight eyes) with subfoveal choroidal neovascularization secondary to angioid streaks were reviewed. Standardized protocol refraction, visual acuity testing, ophthalmologic examinations, color photographs, fluorescein angiograms and indocyanine angiograms were used to evaluate the results of photodynamic therapy with verteporfin. Follow up ranged from 6 to 12 months with a mean (+/- SD) of 8.75 (+/- 2.37) months.Results: Photodynamic therapy with verteporfin was well tolerated in patients with choroidal neovascularization related to angioid streaks. No deterioration in visual acuity was observed. Increase in median best-corrected visual acuity was 1.37 lines (SD +/- 1.59 lines, range 1-5 lines) at the last follow up. Two (25%) patients had no improvement of visual acuity. At the last follow up three (37.5%) patients showed no leakage and three (37.5%) patients had minimal leakage from choroidal neovascularization. Photodynamic therapy related ocular complications were not reported in any case.Conclusions: Photodynamic therapy with verteporfin generally achieved short-term cessation of or decrease of fluorescein leakage from subfoveal choroidal neovascularization without loss of vision in patients with angioid streaks. Further studies with longer follow up are necessary to confirm whether verteporfin therapy is beneficial for subfoveal choroidal neovascularization related to angioid streaks. [ABSTRACT FROM AUTHOR]

Objectives: Behçet's disease (BD) is a chronic inflammatory disorder characterised by aphthous stomatitis, genital ulcerations, erythema nodosum-like manifestations and papulopustular lesions. While a neutrophilic vasculitis accompanies most skin lesions it is usually regarded that the papulopustular lesions in BD are similar to acne vulgaris (AV). The aim of our current study was to further assess the clinical and histopathological features of papulopustular lesions in BD and how these features compared to those of AV., Methods: To analyse the histopathological features of BD and AV, 89 punch biopsies were taken from 58 BD (52 male, 6 female) and 31 AV patients (26 male, 5 female). Sections were evaluated in a blind manner by two different pathologists. A dermatologist who was blind to the patients' diagnosis counted the number of papules, pustules, comedones, folliculitis, cysts and nodules on the face, chest, back, upper and lower extremities., Results: The number of papules, pustules and comedones was higher on the face in the AV group, whereas in the BD group, both number of papules and folliculitis on the back and that of folliculitis were higher on the lower extremities in the AV group. With the exception of comedone formation, which was more frequent among the AV patients [20/31 (64.5%) vs. 23/58 (39.6%), p=0.025] the presence of suppurative folliculitis/perifolliculitis, intrafollicular abscess formation, leukocytoclastic vasculitis or microorganisms were not useful in differentiating BD from AV. However, the interobserver agreement for histologic assessment was low. Kappa was 0.17 for suppurative foliculitis/perifol¬liculitis; 0.39 for intrafollicular abscess formation; 0.51 for leukocytoclastic vasculitis., Conclusions: In the BD group, although the inflammatory lesions located on the face were less than those in the AV group inflammatory lesions such as folliculitis on the legs were only seen, again in the BD group. The papulopustular lesions of BD could not be distinguished from AV by histology. Some of this might be due to high interobsever variation in interpretation. Acne is an inherent manifestation of BD.

Objectives: There had been evidence that low-dose local IFN could be beneficial in the management of recurrent oral ulcers (OUs). We investigated the efficacy and collected initial data on the safety of low-dose natural human IFN-alpha administered by the oral mucosal route in Behçet's syndrome (BS) in a placebo controlled, double blind study., Methods: Eighty-four (59 males and 25 females) patients with BS with mainly skin mucosa disease and a history of recurrent OU for > or = 1 year were studied. When they had at least two OUs with a total diameter of > or = 4 mm, they were randomly allocated to (i) 2000 IFN-alpha IU/day, (ii) 1000 IFN-alpha IU/day and (iii) placebo groups. Subjects were monitored weekly over an initial 4 weeks and bi-weekly for an additional 8 weeks of treatment. OU were counted and measured at each study visit. The primary efficacy end point was the difference in the total ulcer burden at Week 0 compared with that at Week 12., Results: Out of the 84 patients enrolled, 72 completed the trial. There were no statistically significant differences between the treatment arms in terms of the primary endpoint., Conclusions: Low-dose natural human IFN-alpha did not have beneficial effects on reducing the total ulcer burden among BS patients from Turkey. The study also showed that counting the number of ulcers rather than measuring the size would be adequate in future studies., Trial Registration: ClinicalTrials.gov, NCT00483184, http://www.clinicaltrials.gov/ct2/results?term=NCT00483184.