First Description And Other Observations

The first description of the disease is neither by Adamantiades nor by Behget. Hippokrates of Kos (460-377 B.C.) described an illness whose manifestations resembled very well the cardinal signs of Adamantiades-Behget's disease. Already in the 5th century before Christ in his 3rd Epidemion book, case 7 (Fig. 4) he stated But there were also other fevers, as it will be described. Many had their mouths affected with aphthous ulcerations. There were also many defluxions about the genital parts, and...

Materials And Methods

Genomic DNA was extracted from the peripheral blood samples of 191 patients with BD and 325 unrelated healthy control subjects using the QIAamp Blood kit (Qiagen, USA). The disease was diagnosed according to the criteria proposed by International Study Group. Table 1 shows the primers for screening genetic variations for exons 2, 3 and 4 encoding extracellular domains and exon 5 encoding transmembrane region. PCR amplification of exon 2, 3, 4 and 5 were performed using 200 ng DNA, 10 pmol of...

Bes1 Dna In Bd Skin Lesions

The 999 bp fragment of Bes-1 DNA was detected by PCR in mucocutaneous lesions from patients with BD (Fig. 2). The product yielded the expected fragments when digested with Pst-1 restriction endonuclease (Fig. 3). Among the BD patients, 37 were positive for Bes-1 DNA (three of 11 cases) and this was confirmed using skin samples without epidermis (Fig. 3, Table 2). We also demonstrated by PCR the presence of Bes-1 DNA in samples of a genital ulcer (No. 4, incomplete type) and an EN-like eruption...

Patient Profile

A 54-year-old Japanese man suffered from recurrent aphthous stomatitis and pyoderma since January 1989. In 1989, on November 20, he suddenly developed convulsion with subsequent left homonymous hemianopsia. CAT scan and cerebral angiography disclosed hypovascular mass lesion in the right occipital lobe, which reduced in size by treatment with oral corticosteroids. In 1990, he had a series of convulsions. He was admitted to the Department of Neurosurgery, Juntendo University. A diagnosis of...

Patients And Methods

Patients were included by clinical records between 1998 and 2002 at our division. All patients met with international criteria of BD. Lupus anticoagulant (LA) assay was performed by Russell's viper venom time (dRVVT) and dilute APTT (dAPTT). Anticardiolipin antibodies (aCl-beta2-GP I) were assayed with specific EIA for beta2-glycoprotein I (Yamasa kit, Tokyo, Japan). Vascular involvement included deep venous thrombosis, subcutaneous thrombophlebitis, arteritis, and various types of venous...

Diagnosis Investigation And Discussion

Behget's syndrome was diagnosed on the basis of the negative biochemical and immunological results, positive pathergy test, recurrent oral ulcerations, and the presence of both recurrent genital ulcerations and skin lesions. Additional periodontal disease and dysphonic laryngitis were diagnosed (laryngoscopy showed glottis oedema and a 1 -2 mm white lesion on the right ventricular band). On the basis of synchronous symptoms we investigated two associations ear involvement with hearing loss and...

Results

During the course of our initial evaluation, we found eleven patients who had simple recurrent aphthous stomatitis, with no further disease detected. This included eight patients with aphthae minor (simple aphthae minor), two patients with aphthae major, and one patient with herpetiform aphthae. Six patients were excluded who did not have aphthae. These patients included one with Sjogrens Syndrome, two with pemphigus vulgaris, one patient with lichen planus, one patient with factitial disease,...

Prevalence And Distribution

Although BD is suspected to be distributed along the ancient Silk Route as stated above, its prevalence is not known in the areas around the Silk Route of China. Jiang et al.4 demonstrated in 1998 that the highest prevalence districts with 120 patients per 100,000 inhabitants were in the area of the Ningxiahei auto-governed province located in the middle of China where ethnically mixed Aravian and Asian people are living, and in the north area of Heilonyjiang (110 per 100,000) which is near the...

Simple And Complex Aphthosis

The presence of the lesions of RAS is critical to the diagnosis of BD12. The diagnosis is rarely made in the absence of oral aphthosis. Aphthosis can be classified as simple aphthosis or complex aphthosis (Table 1). Patients with BD typically have complex aphthosis. Simple aphthosis is a common, episodic, short-lived type of RAS affecting 20-50 of the population in their youth1,6,7. Complex aphthosis is an uncommon, persistent, chronic type of RAS which may be associated with systemic...

Introduction

Behcet's disease (BD) is a chronic systemic disease which consists of varying combinations of mucocutaneous, ocular, neurologic, cardiovascular, pulmonary, renal, gastrointestinal and other manifestations. Several aspects of the immunopathogenesis are considered but its accurate etiology and pathogenesis are still unknown. The influence of sexual difference on BD is a well-known fact and there are several reports suggesting more severe course of the disease among males1,2. This aspect has never...

The Human Epigenome Project

Disease cannot only be caused by genetic change (e.g. DNA mutation), but also by epigenetic change (e.g DNA modification). The most common epigenetic DNA modification is the methylation of cytosines at CpG dinucleotides. Differential CpG methylation has been shown to play a crucial role in many biological processes such as imprinting, gene regulation, chromatin remodeling, genome stability and, of course, disease (particularly cancer). The Human Epigenome Project aims to catalogue genome-wide...

Hulusi Behcet

Hulusi Behget 1889-1948 Fig. 3 was the first director of the Department of Dermatology and Syphilology of the medical faculty of the University of Istanbul, which was established in 1933. He was named Professor in 193912,13. Between 1918-19 he specialized in dermatology in Budapest Prof. Sellei and Berlin Prof. Arndt . Together with Prof. Braun, Director of the Institute of Microbiology, University of Istanbul, he presented a 34-year-old female patient suffering from recurrent oral aphthous...

References

Yurdakul, S., Gunaydin, I., Tuzun, Y., et al., 1988, The prevalence of Behcet's syndrome in a rural area in northern Turkey. J. Rheumatol. 15 820-822. 2. Yamamoto, S.,I., Toykawa, H., Matsubara, J., et al., 1974, A nationwide survey of Behget's disease in Japan. An epidemiological survey. Jpn. J. Ophthalmol. 18 282-290. 3. Zouboulis Ch.C., 1995, Habilitation thesis, Adamantiades-Behget's disease Clinical and experimental findings in 53 patients from the Berlin area. Berlin. 4. Serdaroglu,...

Treatment

Treatment for ABD is symptomatic and supportive. Medication may be prescribed to reduce inflammation and or regulate the immune system. ABD is initially treated with corticosteroids to suppress acute inflammation. Other therapeutic regimens have to substitute or be added to corticosteroids on a long term basis 16. Many of these drugs can have serious side effects, including liver or kidney damage. Also, patients want to be informed about alternative treatments which sometimes are useless,...

Contents

Initial Contributions of Adamantiades and Behget 3 A Historical Review of Adamantiades-Behget's Disease 7 Multicentre Clinical Registries 17 Epidemiology of Behcet's Disease in Asian Countries and Japan 25 F. Kaneko, K. Nakamura, M. Sato, M. Tojo, X. Zheng, and J.-Z. Zhang Recent Epidemiological Data on Behcet's Disease in Iran 31 F. Shahram, F. Davatchi, A. Nadji, A. Jamshidi, H. Chams, C. Chams, N. Shafaie, M. Akbarian, and F. Gharibdoost Prevalence of Behcet's Disease among Iraqis 37 Current...

Antimicrobial Activity of Synthetic Human CAP18 Peptides to Streptococcus sanguis Isolated from Patients with Behcets

ISOGAI , K.MATUO amp , K. KIMURA , K.YOKOTA , K. OGUMA , M. TOJO , F. KANEKO , S. KOTAKE , and S. OHNO Health Sciences University of Hokkaido Research Division of Innate Immunity Matsuzono Pharmacy, Sapporo Medical University amp National Institute of advanced Industrial Science and Technology Hokkaido Industrial University Okayama University Fukusima Medical University Hokkaido University, Japan

Mayo Patients With Complex Aphthosis

The records of 244 patients with complex aphthosis who had been seen personally by the author were evaluated. There were 152 females 62.3 and 92 males 37.7 . The ages ranged from 15 months to 81 years of age. The peak decade was 20-29 years of age. Patients who suffered oral aphthous ulcerations approximately 50 of the time or had continuous oral ulcerations or had oral and genital ulcerations or suffered major disability from aphthosis were included. Causes for aphthosis were sought for each...

Stage

In each town in Iraq, there is a main food distribution centre, which distributes food rations every month through some agents in the area using the ration ticket system. The rations of the main food items reach every individual within its family at nominal prices which are supplemented by the state. Mouth ulcer questionnaire was distributed to each family with their food ration. Family members aging 16-45 years were registered, which accounts for 14, 155 persons. The questionnaire inquires for...

Disease Activity Index

A disease activity index DAI has to be calculated for each section of each eye, at each visit. The calculation is based upon the inflammatory state of the eye. The severity of the inflammatory indices is graded from 0 to 4. These indices are determined as follows Anterior uveitis AU cells, flare, keratic precipitate and hypopyon in the anterior chamber. Posterior uveitis PU cells, snow ball and snow banking in the posterior chamber. Retinal vasculitis RV periarteritis, periphlebitis, edema of...

Fluorodeoxyglucose positron emission tomography

18Fluorodeoxyglucose positron emission tomography 1SF-FDG-PET as a nuclear medicine scintigraphic technique has been described for functional imaging of neoplastic and inflammatory processes in major organs like the central nervous system, heart, lung, gastrointestinum, and large vessels excluding the urinary bladder and the kidneys. Our experiences with FDG-PET for detection of hypermetabolism as a marker for inflammation in large arterial vessels are pointed out elsewhere in this book9....