The use of interferon-α2a as monotherapy in stage IB patients with mycosis fungoides: A retrospective chart review of patient outcomes.

The aim of this study was to evaluate the response to IFN-α2a treatment as monotherapy in stage IB patients with mycosis fungoides (MF) in second-line therapy. Twenty-five patients with recurrent or persistent MF were included in the study. The diagnosis of MF was established according to clinical and histopathological signs. Clinical staging was made using TNMB classification. IFN-α2a as monotherapy was used as treatment. IFN-α2a was administered at a dose of 3 x 106 units thrice a week subcutaneously as initially described. According to clinical tolerance, the dose was increased every 4 weeks to 6 - 9 x 106 units. IFN-α2a was used more frequently for at least 3 months after complete remission. Treatment success was evaluated with Clinical Response (disappearance of all clinical evidence = Complete Remission [CR], ≥50% decrease in extent or severity = Partial Remission [PR], unresponsiveness to treatment = Stable Disease [SD], progression of MF = Progressive Disease [PD]). The average age was 51.3 ± 9.1. CR and PR were achieved in 11 (44%) and 12 (48%) patients, respectively. PD was observed in two (8%) patients. CR was accomplished at 16.1 ± 9.8 weeks. Recurrences were mostly observed within 1 year (10.4 ± 7.7 months). The recurrence rate was 45.4%. The mean duration of CR was 33.3 ± 7.9 months. Side effects were seen in 36% of the patients (18.2% in CR). The most common side effect was fatigue (12%). The patients received 11 different types of treatment before IFN-α2a treatment. The most frequent therapy prior to IFN-α2a treatment was narrow-band ultraviolet-B (NB-UVB) phototherapy (15 [60%] patients). CR can be achieved in a relatively short period of time in patients receiving IFN-α2a in MF. The duration of CR is reasonable. The side effects of IFN-α2a are acceptable. Therefore, IFN-α2a as monotherapy is a good option in stage IB second-line MF therapy.

A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.

The IL-8 Gene Polymorphisms in Behçet's Disease Observed in Denizli Province of Turkey.

Behçet's disease is a multisystemic inflammatory disorder as a triad of symptoms including recurrent oral and genital aphthous ulceration and uveitis with unknown pathogenesis. IL-8, a proinflammatory cytokine, has been found increased in the active stage of BD. DNA samples were obtained from 88 patients with BD and 112 healthy control subjects in Denizli province of Turkey. All genotyping experiments of SNPs in IL-8 gene were performed using polymerase chain reaction-restriction fragment polymorphism. We found that IL-8 -845 T > C and -738 T > A sites are non-polymorphic. There were no differences in the polymorphisms of IL-8 +396 G/T, +781 C/T, and +1633 C/T sites except IL-8 -251 T > A in between patients and healthy controls. Analysis of IL-8 polymorphisms indicates that the distribution of frequencies seems to be associated with -251 T > A and gender, -251 T > A and erythema nodosum, -251 T > A and ocular involvement, +781 C > T and erythema nodosum, +396 G > T and pathergy positivity, and +1633 C > T and papulopustular lesion. We demonstrated that the frequencies of IL-8 haplotypes were significantly different with BD patients than control group. We found that the distribution of IL-8 haplotypes was significantly different with genital ulcers, ocular involvement, positive pathergy test, erythema nodosum, papulopustular lesions, and arthritis with BD patients than healthy control individuals. Our study suggests that IL-8 gene polymorphisms may affect susceptibility to BD and increase the risk of developing disease. In order to confirm and assess the association of IL-8 and other cytokine gene polymorphisms in the pathophysiology of BD, large cohort studies are needed.

Associations of IL-17 and IL-17 receptor polymorphisms with Behçet's disease in Denizli Province of Turkey.

Although the etiopathogenesis of Behçet's disease is not known, studies conducted in different populations show that it is a multifactorial disease that is thought to develop as a result of the interaction of environmental and genetic factors. IL-17 is thought to induce the neutrophilic inflammation and the tissue damage mediated by immune response in patients. Polymorphisms in the gene region encoding IL-17 and IL-17R molecules may play a critical role in the pathogenesis of the disease and contribute to the elucidation of disease mechanism. We aimed to show the association of IL-17A, IL-17F, and IL-17RC polymorphisms and haplotypes in Behçet's disease patients and its clinical features. We genotyped IL-17A (rs4711998 (A/G), rs8193036 (C/T), rs2275913 (A/G), rs3819025 (A/G), rs8193038 (A/G), rs3804513 (A/T), rs1974226 (C/T), rs3748067 (C/T)); IL-17F (rs763780 (T/C), rs2397084 (T/C)); and IL-17R (IL-17RC) (rs708567 (C/T)) polymorphisms in 88 patients with Behçet's disease and 133 healthy controls using PCR-RFLP-based approach. The results of our study showed that polymorphisms of IL-17A, rs8193036 (C/T), rs3819025 (G/A), rs3804513 (A/T), IL-17F rs2397084 (T/C), and IL-17RC rs708567 (C/T) are associated with the susceptibility to the BD. When the haplotype distributions of all loci of IL-17Aand IL-17A/IL-17F together were examined and in contrast to the data obtained from the controls, the GTGGAACC (27.84%) and GTGGAACCTT (25.57%) have the highest frequencies. In conclusion, the allele and genotype frequency differences of the IL-17A, IL-17F, and IL-17R and haplotype frequencies between Behçet's disease and controls indicate that the genetic structure of Behçet's disease may be different.

Familial Actinic Lichen Planus: Three Cases from the Same Family.

Actinic lichen planus (ALP) that affects sun-exposed areas of the skin is an uncommon variant of lichen planus. While ALP is commonly triggered by ultraviolet radiation exposure, genetic predisposition may also be important in the pathogenesis of the disease. Herein, we report three patients with ALP from the same family, which supports the genetic etiopathogenetic factors of ALP.

A Severe Case of Lymphomatoid Papulosis Type E Successfully Treated with Interferon-Alfa 2a.

Lymphomatoid papulosis (LyP) is a benign papulonodular skin eruption with histologic features of malignant lymphoma. A new variant of LyP which was termed "type E" was recently described with similar clinical and histological features to angiocentric and angiodestructive T-cell lymphoma. LyP type E is characterized with recurrent papulonodular lesions which rapidly turn into hemorrhagic necrotic ulcers and spontaneous regression by leaving a scar. None of the available treatment modalities affects the natural course of LyP. For therapy various modalities have been used such as topical and systemic steroids, PUVA, methotrexate, bexarotene, and IFN alfa-2b. Here we present a severe and devastating case with a very rare variant of LyP type E, which is, to our knowledge, the first case successfully treated with IFN alfa-2a. Now disease has been maintaining its remission status for six months.