Mucocutaneous Lesions of Behçet's Disease

Behçet's disease is particularly prevalent in "Silk Route" populations, but it has a global distribution. The diagnosis of the disease is based on clinical criteria as there is as yet no pathognomonic test, and mucocutaneous lesions, which figure prominently in the presentation and diagnosis, may be considered the diagnostic hallmarks. Among the internationally accepted criteria, painful oral and genital ulcers, cutaneous vasculitic lesions and reactivity of the skin to needle prick or injection (the pathergy reaction) are considered hallmarks of Behçet's disease, and often precede other manifestations. Their recognition may permit earlier diagnosis and treatment, with salutary results. This paper describes the various lesions that constitute the syndrome and focuses on those that may be considered characteristic

C-type lectin domain family 12, member a: a common denominator in Behcet's syndrome and acute gouty arthritis

C-type lectin domain family 12, member A (CLEC12A) is a C-type lectin-like pattern recognition receptor capable of recognizing monosodium urate crystals. Monosodium urate crystals, the causative agents of gout are also among the danger-associated molecular patterns reflecting cellular injury/cell death. In response to monosodium urate crystals, CLEC12A effectively inhibits granulocyte and monocyte/macrophage functions and hence acts as a negative regulator of inflammation. Behcet's syndrome and gout are autoinflammatory disorders sharing certain pathological (neutrophilic inflammation), clinical (exaggerated response to monosodium urate crystals) and therapeutic (colchicine) features. We propose the hypothesis that decreased expression of CLEC12A is a common denominator in the hyperinflammatory responses observed in Behcet's syndrome and gout. Major lines of evidence supporting this hypothesis are: (1) Downregulation/deficiency of CLEC12A is associated with hyperinflammatory responses. (2) CLEC12A polymorphisms with functional and clinical implications have been documented in other inflammatory diseases. (3) Colchicine, a fundamental therapeutic agent used both in Behcet's syndrome and gout is shown to oppose the downregulation of CLEC12A. (4) Behget's syndrome and gout are characterized by a hyperinflammatory response to monosodium urate crystals and other than gout, Behcet's syndrome is the only inflammatory condition exhibiting this exaggerated response. (5) Genomewide linkage and association studies of Behcet's syndrome collectively point to 12p12-13, the chromosomal region harboring CLEC12A. (6) Patients with severe forms of Behcet's syndrome underexpress CLEC12A with respect to patients with mild forms of the disease. If supported by well-designed, rigorous experiments, the forementioned hypothesis pertinent to CLEC12A will carry important implications for therapy, designing experimental models, and uncovering immunopathogenic mechanisms in Behcet's syndrome and gout. (C) 2015 Elsevier Ltd. All rights reserved

İşitme kaybı: Nörobruselloz olabilir mi?

Brusella, insanlara çoğunlukla enfekte yiyeceklerin yenmesiyle veya enfekte hayvan ile doğrudan temas yoluyla geçen bir zoonozdur. Her organ sistemini tutabilir ve buna bağlı olarak çok çeşitli klinik bulgularla ortaya çıkabilir. Çocukluk çağında merkezi sinir sistemi tutulumu nadirdir. Nörolojik bulgular da çeşitlilik gösterebilir ve bu durum tanıyı güçleştirebilir. Burada giderek ilerleyen çift taraflı sensorinöral işitme kaybı ve sonrasında gelişen menenjit ile başvuran bir nörobruselloz vakası sunuldu. On beş yaşındaki kız çocuğuna, klinik bulguları ve pastörize edilmemiş süt ve süt ürünü alımı öyküsüne ek olarak serum ve BOS aglutinasyon testlerinin pozitif sonuç vermesiyle nörobruselloz tanısı konuldu. Brusellozun endemik olduğu bölgelerde çalışan hekimlerin benzer şekilde atipik belirtilerle başvuran hastalarda nörobrusellozu akılda tutması tanıda gecikmelerin önlenmesi açısından önemlidirBrucellosis is a zoonotic infection transmitted from animals to humans mostly by ingestion through infected food products, direct contact with an infected animal. It can involve any organ system and present with a broad spectrum of clinical findings. Central nervous system involvement, so called neurobrucellosis, is rarely seen in pediatric age population. Neurologic manifestations may also show great variability and lead to confusion in diagnosis. Here, we report a case of chronic neurobrucellosis presenting with gradually worsening sensorineural hearing loss and subsequently developed meningitis. A 15 years old girl was diagnosed as neurobrucellosis with the aid of positive serum and CSF agglutination tests, in addition to clinical findings and positive exposure history to unpasteurized dairy products. Clinicians serving in endemic areas should consider brucellosis in differential diagnosis of such atypical clinical presentations to avoid delay in diagnosis and treatment. J Microbiol Infect Dis 2015;5(3): 125-12