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The efficacy and safety of biosimilar infliximab (bio-IFX) was shown in randomised controlled trials and it was approved for all indications of the reference product in several countries. However, a previous case series of 3 patients with Behçet's syndrome (BS) reported disappointing results. We aimed to share our experience with bio-IFX treatment in different types of organ involvement in patients with BS.We reviewed the charts of all BS patients who were prescribed reference infliximab (ref-IFX) or bio-IFX in our BS clinic. Among the 181 BS patients who were prescribed IFX since 2003, 6 (3%) were prescribed bio-IFX due to refractory disease despite conventional immunosuppressives.A total of 6 patients (mean age: 32.1±6.2, mean disease duration: 5.3±1.8 years, 5 men and 1 woman) received bio-IFX for uveitis, nervous system, vascular and joint involvement. Four of the 6 patients obtained remission and stayed in remission during the 16±6.5 months they used bio-IFX. Among the 4 patients who obtained remission, 2 were switched to ref-IFX due to unavailability of bio-IFX infusion set and did not experience adverse events or loss of efficacy. However, relapses occurred during tapering. The other 2 patients are still in remission with bio- IFX. Among the remaining 2 patients, one had to be switched to ref-IFX after the first infusion, due to a change in the reimbursement policy and the other was non-responsive.Our limited experience showed that bio-IFX may be a safe and effective alternative for patients with BS, refractory to conventional immunosuppressives.

Behcet disease is a chronic disease of unknown etiology. It is a systemic vasculitis which can affect many systems and the disease course may be severe in young males. The main aim of treatment is to reduce inflammation and control symptoms of the disease. The main principle of treatment is to prevent attacks of inflammation, reduce symptoms and prevent relapses. Antiinflammatory and immunomodulatory drugs are used for these aims. In this paper, double blinded placebo controlled studies, open studies and case reports have been reviewed to form an update of current treatments. Interferon alpha and TNF antagonists are preferred especially for severe eye involvement. TNF alpha inhibitors have been shown to be effective for neurologic involvement, one of the most severe complications of the disease. Besides these, the guidelines of the Behcet Disease Expert Commission of the European Rheumatism Association (EULAR) have been reviewed. A multidisciplinary approach is necessary to assess the patients’ clinical and laboratory status and prevent side effects of treatment.

Behcet's disease usually begins with cutaneous manifestations, such as recurrent aphthous stomatitis, genital ulcers, erythema nodosum-like lesions, papulopustular findings, and pathergy phenomenon. Recurrent aphthous stomatitis is generally the first sign, and other findings may develop in the course of the disease. There is no specific diagnostic available for Behcet's disease. It is most prevalent among patients along the ancient Silk Road. The high frequency of HLA-B51 among a wide range of ethnic populations favors the role of genetic factors. Behcet's disease usually appears in the third to fourth decade of life, and is rarely seen in children and adults over 50 years of age. It affects both genders equally, but the course of the disease is more severe in men. Eye involvement leading to loss of vision, plus vascular, articular, and central nervous system involvement are more commonly observed among men. Behcet's disease is a systemic inflammatory disorder. A complex genetic background, coupled with innate and adaptive immune system activation, causes the diverse clinical manifestations that characterize the clinical picture.

Background Although genital ulceration with or without scar formation is an important manifestation of Behcet's syndrome, formal data on the rate of genital scarring are lacking. Objective To study prospectively the frequency of genital scar formation after genital ulcers in patients with Behcet's syndrome. Methods One hundred and two patients (72 males and 30 females) with fresh genital ulcers presenting at a dedicated Behcet's syndrome outpatient clinic were followed for 6 weeks. The ulcer location, size, and frequency of scar formation were noted. All patients were treated with similar topical therapy. Results Two hundred and ten genital ulcers were followed in both sexes. The rate of scar formation was 66.2% (102/154) in male patients and 60.7% (34/56) in female patients. When genital ulcers were stratified as a function of size, large genital ulcers (≥ 1 cm in diameter) developed scars more frequently than did smaller ulcers (

Background Intraepidermal IgA pustulosis is a rare vesiculopustular condition characterized by intercellular IgA deposits, neutrophilic infiltration and mild acantholysis. This condition is usually reported in middle-aged or elderly people. We present the clinical, histological and immunofluorescence data of a child with this condition. Observation Our case is an 11-year-old girl who presented a pruritic vesiculopustular eruption involving the trunk, upper extremities, face and oral mucosa. Histopathologic examination revealed an intraepidermal pustule formation composed of neutrophils and some eosinophils and acantholytic cells in the spinal layer. Direct immunofluorescent test showed the deposition of IgA in the intercellular spaces throughout the whole of the epidermis. No circulating IgA anti-intercellular antibodies were detected by indirect immunofluorescence. She was treated with prednisolone 30 mg/daily. After discontinuation of corticosteroid treatment, we did not observe any skin lesions during a follow-up period of 2 years. Conclusion We consider this eruption intraepidermal IgA pustulosis (histologically intraepidermal neutrophilic type) based on clinical and histopathological findings. Intraepidermal IgA pustulosis should be considered in the differential diagnosis of vesiculopustular eruption in childhood.

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes with an unknown etiology. It is a rare disease characterized by overproduction and accumulation of histiocytes within lymph node sinuses and many other extranodal sites, including skin, oral and nasal cavities, respiratory tract, eyelid, and periorbital area. In this case, a 44-year-old woman with diagnosis of Rosai-Dorfman disease, with xanthelasma-like cutaneous lesions on facial area, extending to her neck and acneiform papules on her back, cervical lymph node involvement, and concomitant presence of diabetes insipidus was presented. Histopathological examination of the lesions demonstrated diffuse lymphocyte, plasmocyte, eosinophil, and sparse neutrophil infiltration, together with histiocytes showing phagocytosed inflammatory cells (emperipolesis). Histiocytes demonstrated immunoreactivity with the antibodies for CD68 and S100, whereas they were negative for CD1a and Langerin. Laboratory tests were normal, except mild immunoglobulin G hypergammaglobulinemia. Systemic methylprednisolone therapy was effective for cutaneous lesions.

M Sedef Erdal,1 Gül Özhan,2 M Cem Mat,3 Yıldız Özsoy,1 Sevgi Güngör1 1Department of Pharmaceutical Technology, 2Department of Pharmaceutical Toxicology, Faculty of Pharmacy, 3Department of Dermatology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey Abstract: In topical administration of antifungals, the drugs should pass the stratum corneum to reach lower layers of the skin in effective concentrations. Thus, the formulation of antifungal agents into a suitable delivery system is important for the topical treatment of fungal infections. Nanosized colloidal carriers have gained great interest during the recent years to serve as efficient promoters of drug penetration into the skin. Microemulsions are soft colloidal nanosized drug carriers, which are thermodynamically stable and isotropic systems. They have been extensively explored for the enhancement of skin delivery of drugs. This study was carried out to exploit the feasibility of colloidal carriers as to improve skin transport of naftifine, which is an allylamine antifungal drug. The microemulsions were formulated by construction of pseudoternary phase diagrams and composed of oleic acid (oil phase), Kolliphor® EL or Kolliphor® RH40 (surfactant), Transcutol® (cosurfactant), and water (aqueous phase). The plain and drug-loaded microemulsions were characterized in terms of isotropy, particle size and size distribution, pH value, refractive index, viscosity, and conductivity. The in vitro skin uptake of naftifine from microemulsions was studied using tape stripping technique in pig skin. The drug penetrated significantly into stratum corneum from microemulsions compared to its marketed cream (P

Livedoid vasculopathy (LV) is a chronic, recurrent, painful cutaneous disease with distinctive clinical features and an uncertain etiology. The skin lesions are recognizable by focal purpura, depigmentation and shallow ulcers. Thrombophilic conditions occur frequently in patients with LV. While no definitive treatment exists for LV, smoking cessation, antiplatelet therapy, immunosuppressive treatment, and anabolic steroids are often included in the therapeutic ladder. Recently, a possible link between LV and impaired fibrinolysis was established as cutaneous LV lesions responded to tissue plasminogen activator (t-PA) infusion suggesting that inhibition of the fibrinolysis through plasminogen activator inhibitor-1 (PAI-1) activity may determine the disease course in patients with LV. In this study, we investigated PAI-1 antigen (Ag) and activity levels in 20 patients with biopsy proven LV (mean age 26 ± 11, M/F = 7/13, median disease duration 3.5 years). All patients received antiplatelet treatment with aspirin and/or dipyrimadole and 14 patients received anabolic steroids or immunosuppressive treatment. Fasting PAI-1 Ag and activity levels were measured at 9 AM in all patients. Both Ag (34 (26) ng/ml) (median (interquartile range)) and specific activity (17 (23) IU/fmole) levels of PAI-1 were moderately elevated in LV patients compared to the controls, however, PAI-1 kinetic studies demonstrated markedly enhanced stability of PAI-1 activity in plasma from patients with LV. Specific activity at 16 h was significantly higher than expected specific activity levels (7 (11) vs. 0.07 (0.09) IU/fmole, P < 0.01). While the exact mechanism of increased stability of PAI-1 activity is not known, it may be due to post-translational modifications or increased binding affinity for a stabilizing cofactor. In conclusion, enhanced stability of PAI-1 may contribute to the pathophysiology of LV, and systemic or local treatment with PAI-1 inhibitors may offer a potential treatment alternative in patients with LV.

The diagnosis of Behcet’s disease is based on clinical features. There is no specific diagnostic test. Skin-mucosa lesions like recurrent aphtous stomatitis, genital ulcers, and various cutaneous lesions are its foremost manifestations. The presence of erythema nodosum-like lesions, pseudofolliculitis, papulopustular lesions, acneiform nodules, subcutaneous thrombophlebitis, and cutaneous hyperreactivity (pathergy) are included in the criteria of the International Study Group for Behcet’s Disease even though there is debate that the presence of some of these lesions in any one patient might be coincidental. Skin lesions in Behcet’s disease may differ in both clinical and histopathologic appearance during the disease course and the common underlying pathology is a vasculitis affecting blood vessels of all sizes.

Technetium-99m diaminocyclohexane (DACH) is a new tubular agent excreted via a cationic transport mechanism, like cyclosporine-A (CsA). It is expected that 99mTc-DACH will permit effective assessment of tubular function in CsA-treated patients. To establish the pharmacokinetic characteristics of 99mTc-DACH and to ascertain whether this new agent is useful in CsA-treated patients, 11 healthy volunteers and 15 CsA-treated patients underwent renal imaging and clearance studies using 99mTc-DACH and chromium-51 ethylene diamine tetra-acetic acid (EDTA). 99mTc-DACH yielded satisfactory dynamic renal images in all participants. The mean plasma clearance of 99mTc-DACH was significantly greater than that of 51Cr-EDTA in volunteers (109.4¿19.7 ml/min versus 86.6 +/- 13.7 ml/min, P0.05). However, the urinary excretion of 99mTc-DACH at 90 min was significantly lower than that of 51Cr-EDTA (46.1% +/- 9.3% versus 53.1% +/- 8.6%, P0.05), most probably due to its partial parenchymal retention. The elimination half-life of 99mTc-DACH was significantly increased in CsA-treated patients in comparison to volunteers, and consequently the plasma clearance values were significantly suppressed in these patients, in contrast to 51Cr-EDTA and endogenous creatinine clearance values. In conclusion, our findings indicate that 99mTc-DACH, as a sensitive marker of cationic tubular function, could be used to monitor renal haemodynamics in patients receiving CsA treatment.

Among 50 patients with lepromatous leprosy and borderline lepromatous leprosy in Turkey, the prevalence of HLA-DR2 was 25/50 (50%). The prevalence of the same alleles among 50 healthy controls was 13/50 (26%).