SUMMARYBehçetÕs disease, a systemic vasculitis, can cause varying degrees of activity limitation, fatigue and quality of life impairment. To date, there have been no studies regarding sleep disturbance and its relationship with fatigue and life quality in BehçetÕs disease. We aimed to evaluate sleep disorders and polysomnographic parameters, and to determine their relationship with fatigue and quality of life in BehçetÕs disease. Fiftyone patients with BehçetÕs disease without any neurological involvement were interviewed regarding sleep disorders. Twenty-one subjects with no sleep complaints were included as the control group. Sleep-related complaints were evaluated in a face-to-face interview. Sleep quality, excessive daytime sleepiness, fatigue, depression, anxiety, disease activity ⁄ severity, and quality of life questionnaires and an overnight polysomnography were performed. Prevalences of restless legs syndrome (35.3%) and obstructive sleep apnea syndrome with ⁄ without other sleep disorders (32.5%) were higher than in the control group and the general population. Fatigue was higher in patients with restless legs syndrome and obstructive sleep apnea syndrome, and in those with lower minimum oxygen saturation; hence, only patients with restless legs syndrome had quality of life impairment. Sleep efficiency index and sleep continuity index were lower, and wake after sleep onset, respiratory disturbance index and apnea-hypopnea index were higher than in controls (P < 0.01). Neither sleep disorders nor polysomnographic parameters were related to disease activity and severity. In conclusion, it is important to question sleep disorder followed by a polysomnography, if necessary, in order to improve quality of life and fatigue in BehçetÕs disease. INTRODUC TIONBehçetÕs disease (BD) was first described by Turkish dermatologist Hulusi Behçet in 1937 as a triad of recurrent oral aphthae, genital ulcerations and relapsing uveitis (Behçet, 1937). BD is a chronic, systemic vasculitis with an unknown etiology, which can affect blood vessels (mainly veins) of all sizes. The clinical picture of BD is widely variable. In addition to the mucocutaneous lesions, ocular, vascular, articular, gastrointestinal, urogenital, pulmonary and neurological involvements have been seen during its course, and are characterized by a heterogeneous severity. BD progresses with unpredictable flares alternating with remission periods (Harzallah et al., 2008).The complexity of signs and symptoms in BD can disturb an individualÕs lifestyle by causing limitation in activity. As a result, numerous psychological problems may arise. Furthermore, it was shown in some studies that in patients with BD with fatigue, there was a significant association with impaired quality of life (QoL;Bodur et al., 2006). It was reported that
Histiocytic skin disorders are usually classified as either Langerhans' cell histiocytosis (LCH) or non LCH, based on the pathology. Benign cephalic histiocytosis (BCH) is a rare type of non-Langerhans histiocytitic disorder and is characterized by self-healing multiple small eruptions of yellow to red-brown papules on the face and upper trunk. Histologic features of this disorder show dermal proliferation of histiocytes that have intracytoplasmic comma-shaped bodies, coated vesicles and desmosome-like structures. In this study, we report on a 7-month-old boy who contained small yellow-red papules on his face that spread to his upper trunk. The clinical and histologic features in this patient were consistent with BCH.
Purpose: Behçet's disease is a multisystem disease characterized by recurrent oral and genital ulcers, relapsing uveitis, mucocutaneous, articular, gastrointestinal, neurologic, and vascular manifestations. Paraoxonase is believed to play an important role in protection of LDL and HDL particles from oxidation, in antioxidant effect against lipid peroxidation on cellular membranes, and in anti-inflammatory process. Lipid peroxidation and free oxygen radicals have been thought to play a role in pathogenesis of BD. The association of paraoxonase gene polymorphisms with Behçet's Disease in a group of Turkish patients with clinical manifestations and healthy controls has been investigated. Patients and Methods: Paraoxonase (PON-1-L55M) gene polymorphism was investigated in 50 Behcet patients and 50 healthy individuals with a PCR/RFLP method. Results: There were significant differences between patients and the control group in allele frequencies of the PON1 L55M polymorphism (p=0.04). Also, when patients were compared with the control group according to clinical manifestations, this statistical significance was getting sharper. Compared with the PON55 L allele, the M allele was associated with greater than 3.5 fold (OR 3.5, 95% CI 1.3-8.9) increased risk of ocular (OR 2.4, 95% CI 1.1-5.3), 2.4 fold joint and 3.1 fold (OR 3.1, 95% CI 1.1-8.4) central nervous system manifestations of BD. Conclusion The PON L55M gene polymorphism seemed to play a role in the pathogenesis of BD.
IntroductionPsoriasis is an immune-mediated chronic inflammatory dermatosis. Several studies have shown that patients with psoriasis have a much greater risk of cardiovascular diseases than the normal population. The chronic inflammation observed in psoriasis is thought to have a role in the development of atherosclerosis and vascular endothelial injury.AimTo examine serum pregnancy-associated plasma protein-A (PAPP-A) levels, which has been regarded as a marker of early stage atherosclerosis in patients with psoriasis that do not have concurrent conventional cardiovascular risk markers.Material and methodsForty-one patients diagnosed with a chronic plaque type of psoriasis and 42 equally matched healthy volunteers were included in this study. The PAPP-A levels were compared between patient and control groups and the association between PAPP-A levels and disease duration and severity were evaluated in the patient group.ResultsStatistically, serum PAPP-A levels were significantly higher in the psoriasis group than in the control group (p = 0.015). Serum PAPP-A levels were found to be positively correlated with severity (p = 0.036, r = 0.329) and duration (p = 0.014, r = 0.269) of the disease.ConclusionsAs a marker of early stage atherosclerosis, PAPP-A levels were elevated in the psoriasis group and were correlated with disease duration and severity. This elevation reveals the presence of atherosclerosis in patients with psoriasis. Further studies are needed to confirm the use of PAPP-A as an available and inexpensive screening test and cardiovascular risk assessment for all centers.
ÖzetOfuji'nin papuloeritroderması, deri kıvrımlarını tutmayan eritemli yassı papüllerin görüldüğü, yaygın kaşıntılı erüpsiyonla karakterize bir hastalıktır. Sıklıkla yaşlı erkeklerde görülen hastalığa, lenfadenopati, periferik kanda eozinofili, lenfopeni ve IgE yüksekliği eşlik edebilir. T ve B hücreli lenfoma, internal malignensiler, AIDS, atopik dermatit, psoriasis, ilaç hipersensitivitesi ile ilişkisi rapor edilmiştir. Bu nadir hastalığın tedavisinde topikal ve sistemik kortikosteroidler, psoralen-UVA, etretinat-PUVA, asitretin-PUVA, UVB, siklosporin, interferon α ve azatioprin kullanılmaktadır. Bu yazıda Ofuji'nin papuloeritroderması tanısı koyduğumuz 2 olguyu sunmaktayız. ( AbstractPapuloerythroderma of Ofuji is characterized by a pruritic eruption of widespread, red, flat papules that leads to spare skin folds. The disease occurs primarily in elderly men and is often associated with lymphadenopathy, peripheral blood eosinophilia, lymphopenia and elevated immunoglobulin E levels. Association with T and B cell lymphomas, visceral carcinomas, AIDS, atopic dermatitis, psoriasis and hypersensitivity to drugs have been reported. Topical and systemic corticosteroids, psoralen-UVA, etretinate-PUVA, acitretin-PUVA, UVB, cyclosporin, interferon α and azathioprine have been used in the treatment of this rare disease. In this manuscript, we described two patients with papuloerythroderma of Ofuji. (Turk J Dermatol 2011; 5: 13-7)
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