Rituximab is a beneficial and relatively safe adjuvant treatment for PV that facilitates prolonged clinical remission and has a significant steroid-sparing effect.
BackgroundSoluble CD200 (sCD200) is a novel immuno-effective molecule, which acts to regulate inflammatory and acquired immune responses. Recently, our study group showed that sCD200 was present in serum and blister fluid in a patient with bullous pemphigoid and a patient with toxic epidermal necrolysis. We therefore planned this study to evaluate the sCD200 levels of autoimmune and inflammatory skin disorder patients and to compare them with that of healthy controls.Maleral/MethodsOur study included 30 consecutive patients with psoriasis vulgaris, 15 with pemphigus vulgaris, and 15 healthy controls. Clinical examination and laboratory tests were performed on the same day. Psoriasis patients were also assessed with the Psoriasis Area and Severity Index (PASI) and pemphigus patients were assessed using the Pemphigus Disease Area Index (PDAI). Levels of sCD200 in the serum samples were quantified using ELISA kits.ResultsThe serum sCD200 level was observed to be statistically significantly higher in patients with psoriasis vulgaris (96.7±15.8) compared to patients with pemphigus vulgaris (76.2±14.6), (p<0.001) and healthy controls (26.8±7.0) (p<0.001). The serum sCD200 levels were observed to be statistically significantly higher in patients with pemphigus vulgaris compared with that in healthy controls (p<0.001). In addition, there was a statistically significant correlation between serum sCD200 levels and PDAI (r=0.987, p=0.001). Nevertheless, there was no statistically significant correlation between serum sCD200 levels and PASI (r=0.154, p=0.407).ConclusionssCD200 might play a role in immune response in the pathogenesis of autoimmune and inflammatory skin disorders. However, it remains to be fully elucidated how sCD200 can orchestrate inflammatory response in psoriasis and pemphigus.
Background. Bullous pemphigoid is an autoimmune subepidermal blistering skin disease in which autoantibodies are directed against components of the basement membrane. The disease primarily affects the elderly people and in most of the patients inducing factors cannot be identified. Herein, we report a case of BP that occurred in a patient who was receiving PUVA therapy for the treatment of mycosis fungoides. Main Observation. A 26-year-old woman with mycosis fungoides developed blisters while receiving PUVA therapy. On physical examination tense bullae on the normal skin, remnants of blisters, and erosions were observed on her breasts, the chest wall, and the upper abdomen. Histopathological investigations revealed subepidermal blisters with eosinophilic infiltration and in direct immunofluorescence examination linear deposition of IgG along the basement membrane zone was observed. The diagnosis of bullous pemphigoid was also confirmed by ELISA and BIOCHIP mosaic-based indirect immunofluorescence test. Conclusions. PUVA therapy is an extremely rare physical factor capable of inducing bullous pemphigoid. So the development of blistering lesions during PUVA therapy may be suggestive sign of a bullous disease such as bullous pemphigoid and it should be excluded with proper clinical and laboratory approaches immediately after withdrawal of PUVA therapy.
Amaç: Ülkemizde saç hastalıkları başvurularına ait tanımlayıcı veriler oldukça azdır. Bu çalışmada, mezuniyet öncesi ve sonrası eğitim programlarının oluşturulmasına katkı sağlayacağı düşüncesiyle, bölgemizde gözlenen saç hastalıkları hakkında bilgi sahibi olmak, ünitemize başvuran hastaların özelliklerini tanımlamak ve saç hastalıklarında tanısal yaklaşıma katkı sağlayabilecek verileri belirlemek amaçlanmıştır. Yöntemler: Tanımlayıcı epidemiyolojik çalışmamızda, üniversitemiz Saç Hastalıkları Ünitesi'ne Mayıs 2011-Mayıs 2014 tarihleri arasında başvuran, ardışık 1,617 yeni hastanın verileri geriye dönük olarak değerlendirildi. Bulgular: Genel poliklinik başvuruları arasında saç hastalıkları %7 oranında saptandı. Hastaların yaş ortalaması 32,28±15,45 yaş (0-84 yaş) olup kadın/erkek oranı 2,26 olarak bulundu. Yakınmaların %60 hastada kronik persistan seyir gösterdiği ve hastaların %69,1'inin başvuru anında yakınma sürelerinin yıllarla ifade edildiği saptandı. En sık, androgenetik alopesi (AGA), telojen effluvium ve alopesi areata tanıları konmuştu (sırasıyla; %45,8, %39,4, %17,8). Tanı amacıyla 339 hastada (%20,9) trikoskopi yapılmış ve bunların %85'inde saptanan bulgular tanıya yardımcı olmuştu. Sonuç: Bölgemizde saç sorunları nedeniyle başvuru oranı genel poliklinik sayısı içinde önemli bir miktardadır. AGA, telojen effluvium ve alopesi areatanın iyi tanınması ve muayenede trikoskopi gibi yardımcı yöntemlerin kullanılabilmesi saç hastalıkları alanında başarıyı arttıracaktır. Objective: There are few epidemiological descriptive data on the prevalence of hair diseases in our country. In this study, we aimed to have information about the hair diseases observed in our region, to describe the characteristics of patients admitted to our unit and to supply data for the diagnostic approach which will contribute to developing undergraduate and postgraduate training programs. Methods: In this descriptive epidemiologic study, data of 1.617 consecutive new patients admitted to our University Hair Diseases Unit between May 2011 and May 2014 were analyzed retrospectively. Results: Hair diseases were observed in 7% of the patients who applied to the dermatology outpatient clinic. The mean age of patients was 32.28±15.45 years (0-84 years, range) and female/male ratio was 2.26. Complaints were as chronic persistence in 60% of the patients and 69.1% of the patients described their period of complaint in terms of years at the time of admission. The most frequent hair disorders were androgenetic alopecia (AGA) (45.8%), telogen effluvium (39.4%) and alopecia areata (17.8%). Trichoscopic examination was used in 339 patients (20.9%) and characteristic trichoscopic features contributed to the diagnosis in 85% of them. Conclusion: In our region, admissions due to hair problems have an important ratio among general outpatient clinic visits. For the physicians, being better informed about AGA, telogen effluvium and alopecia areata and being able to perform trichoscopy as the diagnostic modality will increase the success in the diagnosis ...
Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease of the skin and mucous membranes. Although there is some evidence pointing towards a genetic predisposition by some human leukocyte antigen (HLA) genes, familial occurrence of PV is very rare. Most of the familial PV cases so far reported have been in mother and daughter and in siblings. PV in father and son, as presented here, has not been reported in the literature before, except an unconfirmed report. The diagnosis of PV was established by histologic, cytologic studies and enzyme linked immunosorbent assay (ELISA) in Case 1 and by ELISA and BIOCHIP indirect immunofluorescence test in Case 2. The son was responsive to moderate doses of methylprednisolone, with the treatment continuing with tapered doses. The father was in a subclinic condition; consequently, only close follow-up was recommended. HLA typing studies revealed identical HLA alleles of HLA-DR4 (DRB1⁎04) and HLA-DQB1⁎03 in both of our cases; this had been found to be associated with PV in prior studies. Familial occurrences of PV and related HLA genes indicate the importance of genetic predisposition. The first occurrence of confirmed familial PV in father and son is reported here.
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