We present an extremely rare case of Woringer-Kolopp disease with CD4+/CD8+ immunohistochemical features in a 27-year-old male patient, which involved the right gluteal region continuing down to the lower thigh. Although the lesions had been present for 15 years, the disease abruptly changed its indolent clinical behavior in the last 3 months and gained local aggressiveness without features of dissemination. The patient was successfully treated with 6 MeV electron-beam radiotherapy and the radiation portal was determined to be the gross lesion plus 1-cm margins around the lesion.
View Article and Find Full Text PDFNeurofibromatosis type 1 (NF-1) is the most frequently seen form of neurofibromatosis. The characteristic features of this disorder are café au lait macules, neurofibromas, axillary and inguinal freckling, Lisch nodules, bone lesions such as sphenoid dysplasia, and optic glioma. Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rarely seen disease characterized by complete vaginal agenesis and uterine aplasia/hypoplasia.
View Article and Find Full Text PDFUnlabelled: LABELED BACKGROUND: Radiotherapy may cause common cutaneous side effects such as acute and chronic radiodermatitis and also rare skin conditions.
Case Report: A 42-year-old woman who received radiotherapy for invasive breast carcinoma developed multiple, scaly, rough papules and nodules restricted to treatment fields six months after completion of the treatment. A relapse of the breast cancer and/or seborrheic keratosis was ruled out by histological confirmation of verruca vulgaris.
Juvenile hyaline fibromatosis (JHF) is a rare, autosomally-recessive disease characterized by papulonodular skin lesions, soft tissue masses, joint contractures, gingival hypertrophy and osteolytic bone lesions. Its onset is in infancy or early childhood. The most commonly affected sites are the nose, chin, ears, scalp, back and knees.
View Article and Find Full Text PDFA 17-year-old girl presented with multiple, painful, erythematous blisters and ulcerated lesions on the shins and buttocks. She also had arthralgia. She had suffered from juvenile rheumatoid arthritis (JRA) and received anti-inflammatory agents and oral glucocorticoids for eight years.
View Article and Find Full Text PDFWe report a case of toxic epidermal necrolysis who was successfully treated with intravenous immunoglobin and granulocyte colony-stimulating factor. He had poor prognostic factors such as extensive epidermal loss, neutropenia, acute respiratory distress syndrome and candida sepsis, but nonetheless made a complete recovery.
View Article and Find Full Text PDFErythema dyschromicum perstans (EDP) is a rare disorder characterized by asymptomatic, slowly progressive, ash-gray macular pigmentation of the skin, which usually occurs from age 5 years through adult life. Most cases reported to date are of Latin American and Indian patients. Rare cases have been reported from Turkey.
View Article and Find Full Text PDFObjective: To determine the effect of plasma from patients with Behçet's disease (BD) on the production of nitric oxide (NO) in cultured human umbilical vein endothelial cells (HUVECs).
Subjects And Methods: NO levels were measured in cell culture media after 24-hour incubation of the cells with plasma obtained from 22 BD patients and 16 age/sex-matched healthy control subjects. After treatment of the patients with colchicine and/or nonsteroidal anti-inflammatory drugs, 12 of the patients were considered to be in the inactive phase of the disease.
Background: Hyperhomocysteinemia may constitute an independent risk factor for cardiovascular disease and may promote atherothrombosis. Psoriasis is one of the diseases associated with increased atherothrombosis. The aim of the present study was to examine serum total homocysteine (tHcy) level and its relationships with atherothrombotic markers.
View Article and Find Full Text PDFBackground: Psoriasis is a common chronic and recurrent inflammatory skin disease that can occur due to abnormalities in essential fatty acid metabolism, lymphokine secretion, free radical generation, lipid peroxidation and eicosanoid metabolism, and has been associated with increased frequency of cardiovascular events. The current study was designed to evaluate plasma lipids, susceptibility of LDL to oxidation and oxidant-antioxidant status and their relationships in patients with psoriasis.
Methods: The study group included 35 patients with psoriasis (18 females and 17 males), and 35 sex- and age-matched healthy volunteers (16 females and 19 males).
Objectives: Behçet's disease is a multisystem disorder characterized by a chronic inflammation including acute attacks and remission periods. Decreased enzyme activity of the antioxidant system and increased levels of free radicals may have important roles in the damage of tissues observed in the disease period. In addition, the atherogenic tendency of serum lipid, lipoproteins, lipid peroxidation levels and endothelial dysfunction accompany the above mentioned findings.
View Article and Find Full Text PDFJ Eur Acad Dermatol Venereol
September 2000
Congenital primary hypoparathyroidism is very rare in infancy. It may be isolated or associated with other developmental defects, arising from the third and fourth pharyngeal pouches such as DiGeorge syndrome. Initial symptom of isolated primary hypoparathyroidism in an infant is usually generalized convulsion due to hypocalcemia.
View Article and Find Full Text PDFPsoriasis is associated with changes in plasma lipid and lipoproteins, which may play a role in the development of occlusive vascular disease. The oxidation of low-density lipoprotein (LDL) is considered a key event in the development and progression of atherosclerosis. Autoantibodies against oxidized LDL (auAb-oxLDL) may contribute to understanding the relationship between oxidative processes and development of atherosclerosis.
View Article and Find Full Text PDFBackground: Change of lipids and lipoprotein metabolism and an imbalance of the oxidant-antioxidant system related to the disease activity have been reported in Behçet's disease. Therefore, there is a tendency of oxidative modification of lipids and lipoproteins in patients with the disease.
Objective: To investigate serum autoantibodies against oxidatively modified low-density lipoprotein (oxLDL) as a marker for the degree of in vivo oxidation of lipoproteins in Behçet's disease.
Background: Behçet's disease is a chronic multisystemic disorder which is characterized by a relapsing systemic inflammatory process. One of the features of Behçet's disease is endothelial dysfunction resulting from many factors.
Objective And Methods: We conducted this study to show if there is any change of plasma nitrite and nitrate levels by using the enzymatic-spectrophotometric method as an indicator for nitric oxide (NO) production, considering the disease activity in patients with Behçet's disease.
Behçet's disease is a chronic multi-systemic disorder which is characterized by a relapsing systemic inflammatory process. The alteration of lipid profile and lipid peroxidation resulting from the inflammatory process may he associated with an increased risk for atherosclerosis in patients with Behcet's disease. We investigated lipids, lipoprotein and lipid peroxidation and their inter-relationships considering the disease activity.
View Article and Find Full Text PDFAlthough some increased enzyme activities in PMN leukocytes isolated from the circulation of psoriatic patients have been previously described, none of these enzymes had been studied in plasma for determination of disease activity. The purpose of this study was to evaluate whether plasma PMN elastase levels might be a good marker for determination of disease activity of psoriatic patients. Plasma PMN elastase and some acute phase reactants including alpha-1 antitrypsin, alpha-2 macroglobulin, fibrinogen, erythrocyte sedimentation rate(ESR), and transferrin were studied in thirty-nine patients with psoriasis, including 16 controlled patients, and forty healthy control subjects.
View Article and Find Full Text PDFObjectives: To investigate whether plasma levels of Lp(a) lipoprotein (Lp(a)) are predictors of defective fibrinolytic activity, leading to thrombosis, in patients with Behçet's disease.
Methods: Plasma Lp(a) was measured by enzyme linked immunosorbent assay, lipids and lipoproteins by enzymatic methods, and apolipoproteins A-I and B, fibrinogen (turbidimetric method), tissue plasminogen activator (t-PA), plasminogen activator inhibitor-1 (PAI-1), and D-dimer levels by enzyme immunoassay. Their levels and interactions were evaluated in 33 patients with Behçet's disease (including five with thrombotic complications) and 30 healthy control subjects.