Epidemiology of pemphigus in Turkey: One-year prospective study of 220 cases

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation).  The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.   </p

Clear cell sarcoma

Malignant melanoma (MM) of soft tissue, also called clear cell sarcoma (CCS) of tendons and aponeuroses, derives from the neural crest. CCS is similar morphologically to MM but has no precursor skin lesion, and instead, has a characteristic chromosomal translocation. Prognosis is related to the tumor size. Early recognition and initial radical surgery is the key to a favorable outcome. The tumor has to be differentiated from other benign and malignant lesions of the soft tissues, such as fibrosarcoma. The demonstration of melanin and a positive immunohistochemical reaction for S-100 protein and HMB-45 can assist in the differential diagnosis. We report the case of a 58-year-old woman with CCS arising from the soft tissue of her little finger

Multiple sub-epidermal calcified nodule mimicking eruptive xanthoma: A case report and review of the literature

Sub-epidermal calcified nodule (SCN) is an uncommon form of idiopathic calcinosis. It usually occurs in children, particularly in the head and neck region, presenting as a solitary, painless, yellow-white nodule with papillomatous features. These lesions occur twice as common in males compared with females. The pathogenesis is uncertain, but the clinical and histological features of this lesion are distinctive. We report a case of 22-year-old man with multiple nodules bilaterally located on the dorsum of hands simulating eruptive xanthoma. Histopathological examination of one of the excised lesion confirmed the diagnosis showing epidermal and sub-epidermal deposition of calcium. This paper presents a review of the literature and adds a new case of SCN

Genital ulcerative pyoderma gangrenosum in Behçet′s disease: A case report and review of the literature

Behçet′s disease (BD), first described by Hulusi Behcet, is a multisystemic disease characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis and vascular disease. Pyoderma gangrenosum (PG) is a rare, chronic, sterile pustular and progressive ulcerative process of unknown cause; sometimes can participate in the differential diagnosis of Behcet′s ulceration. A 33-year-old woman complained a severe genital ulcer. She had a purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm. A punch biopsy at ulcer margin showed that the lymphocytic panniculitis was extending to the subcutaneous fat tissue without fibrin deposition or necrotic changes in the vessel wall. Based on the clinical and histological findings, she was diagnosed as genital ulcerative PG, which occurred during the exacerbation of BD

Markers of Endothelial Dysfunction and Evaluation of Vascular Reactivity Tests in Behcet Disease

KISA, Ucler/0000-0002-8131-6810; Gunduz, Ozgur/0000-0003-1021-5219;WOS: 000346181200014PubMed: 24277913We assessed endothelial dysfunction (ED) in patients with Behcet disease (BD; n = 40) and healthy controls (n= 20). Serum lipid, homocysteine, asymmetric dimethylarginine (ADMA) and high-sensitivity C-reactive protein (hsCRP) levels, erythrocyte sedimentation rates (ESRs), and ultrasonographic flow-mediated dilatation (FMD) were measured. Mean hsCRP, ESR, homocysteine, and ADMA were significantly higher in the BD group (P <.001 for all). Patients with active BD had higher serum levels of hsCRP, homocysteine, and ESR compared with those in remission (P <.001, P <.001, and P =.005, respectively). Flow-mediated dilatation was significantly lower in patients with BD than in controls (P =.001). Flow-mediated dilatation correlated negatively with BD duration and serum ADMA levels (P <.001, r =-.745 and P <.001, r = -.682); a positive correlation was seen between serum ADMA levels and BD duration (P <.001, r =.552). Only stepwise multivariate regression analysis revealed BD duration to have a significant effect on FMD. Flow-mediated dilatation, in conjunction with markers of inflammation, may evaluate ED in patients with BD.Kirikkale University Research Project UnitKirikkale University [2008-16]The author(s) disclosed receipt of the following financial support for the research, authorship and/or publication of this article: Our study was supported by Kirikkale University Research Project Unit. The Project number: 2008-16