[Analysis of clinical diagnosis and treatment of Behet's disease with sore throat as the first symptom].

Objective:To summarize the clinical and diagnostic characteristics of Behet's disease with sore throat as the first symptom, so as to improve clinicians' understanding of the disease. Methods:The clinical data of 28 patients with Behet's disease who were first treated in Department of Otorhinolaryngology, Peking Union Medical College Hospital from January 2015 to January 2022 were analyzed retrospectively. All patients were examined by electronic laryngoscopy at the first visit and treated with local symptomatic therapy. Twenty-one patients were examined for C-reactive protein and blood sedimentation, and some patients were examined for immunological parameters. After the diagnosis of leukoaraiosis was confirmed, treatment was based on immunosuppression and could be supplemented with oral hormone therapy depending on the extent of the lesion and other organ involvement. Results:Sore throat was the first symptom of all patients, which could be accompanied by dysphagia or dyspnea. Endoscopy revealed oropharyngeal ulcer in 25 cases（89.3%）, laryngeal ulcer in 15 cases（53.6%）, hypopharyngeal ulcer in 14 cases（50.0%）, and adhesion of nasopharynx or throat in 8 cases（28.6%）. All patients had oral ulcer at the same time, including 21 cases（75.0%） with genital ulcer, 18 cases（64.3%） with skin damage, and 11 cases（39.3%） with eye disease. In the course of disease, there were 4 cases（14.3%） with joints involvement, 3 cases（10.7%） with nervous system disease, and 1 case（3.6%） with digestive system disease. The treatment is mainly immunosuppressive, and oral hormone therapy can be supplemented according to the extent of the disease and the involvement of other organs. Conclusion:The clinical manifestations of Behet's disease, with sore throat as the first symptom, are diverse. The ulcer may involve multiple parts of the throat, and a few patients may involve multiple systems of the whole body. Clinical attention should be paid to the early diagnosis and timely treatment to prevent or delay the involvement of the lesion in important organs or systems.

Chinese consensus on diagnosis and treatment of intestinal Beh?et′s disease / 中华消化杂志

Beh?et′s syndrome is a kind of chronic systemic vasculitis with involvement of multiple organs. Intestinal involvement of Beh?et′s syndrome is presently named as intestinal Beh?et′s syndrome. Recently, there is considering another kind of disease type with only typical intestinal ulcers. Since it is difficult to differentiate intestinal Beh?et′s syndrome from Crohn′s disease, intestinal tuberculosis, intestinal lymphoma, and intestinal manifestations of many other autoimmune diseases, and there is limited evidence for the therapy of intestinal Beh?et′s syndrome, proposing diagnosis and treatment recommendations for intestinal Beh?et′s syndrome through evidence-based judgment will be of great significance for clinical practice.

Clinical characteristics of 47 cases of intestinal Beh?et′s disease / 中华消化杂志

Objective:To summarize the clinical features of intestinal Beh?et′s disease, so as to provide reference for the diagnosis of the disease.Methods:From April 1 2014 to January 31 2019, the clinical data of 47 patients diagnosed as intestinal Beh?et′s disease at the Second Hospital of Hebei Medical University were retrospectively analyzed, which included initial symptoms, gastrointestinal symptoms, complications, erythrocyte sedimentation rate (ESR), the levels of C reactive protein (CRP), hemoglobin, serum albumin, results of acupuncture test, gastrointestinal involved site and ulcer shape. At the same time, gender differences of clinical manifestations were compared. Chi-square test was used for statistical analysis.Results:Among 47 patients with intestinal Beh?et′s disease, the initial symptoms of 26 (55.3%) cases were gastrointestinal symptoms. Abdominal pain was the most common symptom, the others were diarrhea, anorexia, abdominal distension and perianal abscess, and the incidence rate was 80.9%(38/47), 46.8% (22/47), 42.6% (20/47), 36.2% (17/47) and 2.1% (1/47), respectively. The main complications were gastrointestinal bleeding, perforation and obstruction, and the incidence rates was 40.4% (19/47), 4.3% (2/47) and 4.3% (2/47), respectively. Thirty-seven (78.7%) patients had different degrees of hypoalbuminemia (serum albumin<35 g/L). The CRP level of 36(76.6%) patients increased. The ESR of 36 (76.6%) patients increased. Twenty-two (46.8%) patients had mild anemia (hemoglobin<90 g/L). The acupuncture test was positive in 25 (53.2%) patients. The involved sites of gastrointestinal tract were terminal ileum and ileocecal junction, colon, esophagus, duodenum and jejunum, stomach, and rectum, the proportion was 57.4% (27/47), 27.2% (13/47), 23.4% (11/47), 23.4% (11/47), 17.0% (8/47) and 8.5% (4/47), respectively. All 47 (100.0%) patients had oral ulcers. 62.1%(18/29) patients presented with multiple ulcers under endoscope. The shape of ulcer was round ulcer, irregular ulcer, and longitudinal ulcer, the proportion was 48.3% (14/29), 34.5% (10/29) and 17.2 (5/29), respectively. The incidence rate of genital ulcer of female patients with intestinal Beh?et′s disease was higher than that of male patients with intestinal Beh?et′s disease (85.7%, 18/21 vs. 30.8%, 8/26), and the difference was statistically significant ( χ2=14.189, P<0.01). There were no significant differences between the female group and the male group in the incidence rate of oral ulcer, abdominal pain, diarrhea, and positive rate of acupuncture test (100.0%, 21/21 vs. 100.0%, 26/26; 85.7%, 18/21 vs. 76.9%, 20/26; 42.9%, 9/21 vs. 50.0%, 13/26; 52.4%, 11/21 vs. 58.3%, 14/26, all P>0.05). Conclusions:The common clinical symptoms of intestinal Beh?et′s disease are oral ulcers, abdominal pain, diarrhea and genital ulcer. Female patients with intestinal Beh?et′s disease are more likely to develop genital ulcer than male patients with intestinal Beh?et′s disease. Multiple ulcers are more common under endoscopy, which are round ulcer, irregular ulcer and longitudinal ulcer. The most common sites are the terminal ileum and ileocecal junction, followed by colon, esophagus and other parts.

Serum Metabolomic Profiling of Patients with Non-Infectious Uveitis.

The activities of various metabolic pathways can influence the pathogeneses of autoimmune diseases, and intrinsic metabolites can potentially be used to diagnose diseases. However, the metabolomic analysis of patients with uveitis has not yet been conducted. Here, we profiled the serum metabolomes of patients with three major forms of uveitis (BehÒ«et's disease (BD), sarcoidosis, and Vogt-Koyanagi-Harada disease (VKH)) to identify potential biomarkers. This study included 19 BD, 20 sarcoidosis, and 15 VKH patients alongside 16 healthy control subjects. The metabolite concentrations in their sera were quantified using liquid chromatography with time-of-flight mass spectrometry. The discriminative abilities of quantified metabolites were evaluated by four comparisons: control vs. three diseases, and each disease vs. the other two diseases (such as sarcoidosis vs. BD + VKH). Among 78 quantified metabolites, 24 kinds of metabolites showed significant differences in these comparisons. Four multiple logistic regression models were developed and validated. The area under the receiver operating characteristic (ROC) curve (AUC) in the model to discriminate disease groups from control was 0.72. The AUC of the other models to discriminate sarcoidosis, BD, and VKH from the other two diseases were 0.84, 0.83, and 0.73, respectively. This study provides potential diagnostic abilities of sarcoidosis, BD, and VKH using routinely available serum samples that can be collected with minimal invasiveness.

Beh(c)et's disease complicated with meningeal thickening: a case report and literature review / 中华风湿病学杂志

Objective To explore the characteristics of diagnosis and treatment of Behset's disease (BD) with meningeal thickening and to improve doctors" awareness of such diseases.Methods We reported a missed diagnosis of a patient with BD complicated with diffuse meningeal thickening,and reviewed the related literature.Results A 25-year-old young man,manifested mainly as recurrent headache,fever,recurrent oral ulcer,erythema skin nodules and folliculitis,and his cranial radiology revealed diffuse meningeal thickening and intracranial venous sinus thrombosis.We diagnosed him as neuropathy associated with BD.After the treatment with steroids,cyclophosphamide,infliximab and anti-coagulants,his symptoms improved rapidly.Conclusion In clinical,BD complicated with meningeal thickening is rare,which is easily misdiagnosed or miss-diagnosed.For the patients with unexplained meningeal thickening,the symptoms of BD,such as recurrent oral or genital ulcers,ophthalmitis and skin lesions,should be acquired in detail.In addition to steroids and immunosuppressive agents,anti tumor necrosis factor (TNF)-alpha can also be used in the treatment of BD with meningeal thickening.

Biological agents in the treatment of severe and/ or refractory neuro-Beh(c)et's disease / 中华风湿病学杂志

Objective To explore the use of biological agents in Neuro-Beh(c)et's disease (NBD).Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016.The continuous variables were analyzed by t test.Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD).Their age at neurological onset was (31±12) years old.Four cases presented with multiple lesions.The brainstem,spinal cord,cerebral hemisphere and cerebellum involvement were presented in 4,3,3 and 2 patients,respectively.The Rankin score at the onset of NBD was (4.0±0.7).The biological agents were administrated when corticosteroids and immunosuppressant were ineffective.Three cases received tumor necrosis factor (TNF)-α inhibitors therapy,among whom one patient had gastrointestinal ulceration.One patient with refractory retinal vasculitis received interferon-α therapy.One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid.All patients achievcd clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81,P＜0.01) after the treatment with biological agents.The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases,indicating a potential steroid and immunosup-pressant-sparing effect.No serious adverse events were observed during the follow-up.Conclusion Neurological involvement is a severe complication of Behqet's disease.We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD.

The 458th case: fever, cytopenia, abdominal pain, and multiple intestinal ulcerations with perforation / 中华内科杂志

Trisomy 8 in myelodysplastic syndrome (MDS) plays an important role in concurrent intestinal Beh?et's disease (BD) pathogenesis.Here,we reported a case of intestinal BD combined with MDS involving trisomy 8.A 48-year-old woman who has had a 20-year history of recurrent oral ulcer,perineal ulcer and iris,was diagnosed as MDS with trisomy 8 four years ago.She developed high fever and acute abdominal pain.Multiple ulcerative perforations in ileum and colon were found by endoscopy,meeting the criteria for intestinal BD.The patient was successfully treated with immunomodulatory drugs.

The 458th case: fever, cytopenia, abdominal pain, and multiple intestinal ulcerations with perforation / 中华内科杂志

Trisomy 8 in myelodysplastic syndrome (MDS) plays an important role in concurrent intestinal Beh?et's disease (BD) pathogenesis.Here,we reported a case of intestinal BD combined with MDS involving trisomy 8.A 48-year-old woman who has had a 20-year history of recurrent oral ulcer,perineal ulcer and iris,was diagnosed as MDS with trisomy 8 four years ago.She developed high fever and acute abdominal pain.Multiple ulcerative perforations in ileum and colon were found by endoscopy,meeting the criteria for intestinal BD.The patient was successfully treated with immunomodulatory drugs.

Clinical analysis of 25 cases of Beh?et′s disease complicated with pulmonary hypertension / 中华全科医师杂志

Objective To understand the clinical features of Behcet′s Disease( BD) with pulmonary hypertension( PH) .Methods The etiology, clinical features, treatment and prognosis of 25 patients with Bahcet′s disease ( BD) complicated with pulmonary hypertension ( PH) admitted in Peking Union Medical College Hospital from January 2000 to August 2015 were retrospectively reviewed.Results Total 912 BD patients were hospitalized during the same period, among whom 25 cases were complicated with PH accounting for 3%.There were 15 males (60%) and 10 females (40%), with the mean age of (33 ±12) years (range 19 to 66 years).The median interval from the diagnosis of BD to the onset of PH was 1 year (range 0 to 40 years).The most common cause of PH was heart valve disease (n=10, 40%), followed by pulmonary arterial stenosis or occlusion (n=6, 24%), pulmonary arterial aneurysm with thrombus (n=1, 4%), pulmonary thrombosis (n=1, 4%), cardiomyopathy (n=1, 4%);there were no causes identified in 6 cases (24%).Pulmonary arterial pressures estimated by echocardiography were 40 to 117 mmHg (1 mmHg=0.133 kPa) with a mean pressure of (60 ±22) mmHg.When the PH developed, elevated erythrocyte sedimentation rate ( ESR ) and increased hypersensitive C-reactive protein ( hs-CRP ) were founded in 48% ( 11/23 ) and 82% ( 14/17 ) of patients, respectively.After treated with glucocorticoid (96%,24/25), immunosuppressive agents(92%, 23/25), anticoagulation or thrombolysis(36%,9/25) and specific targeted vasodilator(32%,8/25), the levels of ESR and hs-CRP declined in 91% (10/11) and 71%(10/14) of patients, respectively; and pulmonary arterial systolic pressure declined in 50% of cases (8/16).Among 9 patients followed for 2 to 96 months, 4 died, 1 aggravated, 2 kept stable and 1 improved.Conclusion PH is an uncommon complication in disease.Heart valve disease, pulmonary artery involvement are the major causes of PH.The therapeutic effect and prognosis are poor.

Progress and prospect of treatment for ocular Beh(c)et disease / 中华实验眼科杂志

Ocular Beh(c)et disease,which usually presents as panuveitis,with characteristic of vision impairment and relapse,is one of the most refractory uveitis entities.Usually,one or more conventional immunosuppressive agents,such as glucocorticoid,ciclosporin,chlorambucil,cyclophosphamide,etc,are needed in disease management.However,long-term use of immunosuppressive agents would result in severe adverse effects.New biologics,such as interferon,antitumor necrosis factor agents,interleukin or its receptor antagonists,provide new therapeutic approaches to the treatment for this disease.This paper reviewed the conventional therapies and biologics applied in ocular Beh(c)et disease.

Cerebral venous sinus thrombosis in Beh(c)et's disease: clinical study and follow-up of 12 cases / 中华风湿病学杂志

Objective To investigate the clinical characteristics of Beh(c)et's disease (BD) complicated with cerebral venous sinus thrombosis (CVST).Methods In patients data at Peking Union Medical College Hospital between February 2004 and April 2013 were reviewed retrospectively to identify patients who were diagnosed as BD complicated with CVST.SPSS 19.0 software was used for statisticals analysis.Data of normal distribution was expressed by x±s (standard deviation) and that of abnormal distribution by median and range.Results Of 601 inpatients with BD during that period,12 patients (2％,6 male and 6 female) developed CVST,and their age was (28±10) years old (range 15 to 52 years old).Most of patients (92％,11/12)suffered neurological symptoms after other initial systemic symptoms of BD in mean (56±35) months.The most frequent neurological signs were headache (11 patients,92％),papilledema (9 patients,75％),nausea/vomiting (8 patients,67％) and blurred vision (6 patients,50％),with progression as the main mode of onset (9/11,82％).Eight out of 10 patients (80％) were found elevated cerebrospinal fluid (CSF) pressure by lumbar puncture.Occlusions of multiple sinuses (8 patients,67％) were more frequent than single occlusion (4 patients,33％).The most frequent locations of CVST were the transverse sinus (9 patients,75％),superior sagital sinus (8 patients,67％) and sigmoid sinus (7 patients,58％).Other than CVST,8 patients were complicated with other sites of thrombosis (7 cases in lower extremity veins and 1 in pulmonary artery).In addition,cerebral infarction was found in 3 patients,subarachnoid hemorrhage and right temporal lobe hemorrhage in 1 patient,respectively.After glucocorticoid,immunosuppressant,as well as anticoagulant and dehydration therapy,with a median follow-up of 8 months (0.7 to 88.8 months),10 patients achieved clinical improvements (83％),2 patients relapsed (17％).Conclusion CVST is a relatively rare complication of Beh(c)et's disease,with headache,papilledema,nausea/vomiting and blurred vision as the common clinical manifestations.By early identification,timely diagnosis and active treatment,the prognosis of most patients is good.

A Meta analysis of association between IL-1 gene polymorphisms and Beh(c)et disease susciptibility / 中华实验眼科杂志

Background Interleukin (IL)-1a-889C/T,IL-1β-S11C/T,+ 3962C/T and IL-1Ra-2 are different morphology of single nucleotide of interleuki-1 (IL-1).The potential relationship of IL-1 polymorphisms with Beh(c)et disease has been studied in several populations and groups.However,study outcomes are controversial for a long duration.Objective This Meta-analysis was to determine whether the IL-1 gene polymorphisms is associated with the pathogenesis and development of Beh(c)et disease.Methods The literature of the assoiation between IL-1 polymorphisms and Beh(c)et disease was retrieved from Medline,EMBASE,Cochrane Library,Web of knowledge,Google Scholar,Wanfang and CNKI databases.All the published original articles (case-control studies) were reviewed with the deadline May 31,2013,and the languages were limited to English and Chinese.Included reports were evaluated based on Newcastle-Ottawa Scale (NOS) score.The potential influence of IL-1α-889TT gene,IL-1β-3962C,IL-1β-511T and IL-1Ra-2 polymorphisms on Beh(c)et disease were analyzed.RevMan5.0,the Cochrane collaboration software program,was used to prepare and complete this review.The effect size was assessed using fixed effect model in the index with lower heterogeneity (I2 ＜50％) or randomized effect model in the index with higher heterogeneity (I2＞50％).Results The literature search resulted a total of 370 cases-controlled studies,and 7 studies met the included criteria,with the NOS scores ≥ 8.The total patients were 499 and normal controls were 708.Meta-analysis was performed on several populations.Overall,the polymorphisms of IL-1β-3962C allele increased the susciptibility of Beh(c)et disease (OR =1.41,95％ CI:1.06-1.88,P =0.02),and IL-1α-889TT genotype reduced the risk of Beh(c)et disease (OR =0.61,95 ％ CI:0.40-0.92,P =0.02).However,There was no significant association between variants of IL-1β-511T allele (OR =0.84,95％ CI:0.58-1.23,P=0.38) or IL-1RA-2 allele (OR =1.25,95％ CI:0.50-3.14,P=0.63) and Beh(c)et disease susciptibility.Conclusions These results suggest that Beh(c)et disease is associated with the IL-1 gene polymorphisms at the locations α-889C/T and β-3962C/T.A larger sample size clinical data still are need to confirm this conclusion.

Morbidity of solid cancer in Behςet's disease: analysis of 11 cases in a series of 506 patients.

PURPOSE: Behςet's disease (BD) is rarely reported to be associated with malignancies in the literature. However, the frequency of cancer in BD patients remains unknown. This study evaluated cancer morbidity in BD patients compared with that in the general population of Korea. MATERIALS AND METHODS: A retrospective chart review was performed on 506 patients visiting our hospital from 1994 to 2011 for BD. We analyzed the standardized morbidity rate (SMR), which is the ratio of observed to expected malignancies. Furthermore, we reviewed cases of solid cancer in BD patients in the literature. RESULTS: Of the 506 patients with BD, 11 (2.17%) developed cancer. We found a variety of solid cancers without predominance and no hematologic malignancies. The total number of cancers observed was less than expected, which was determined from the statistical data of the National Cancer Information Center of Korea, with an SMR of 0.023 (95% confidence interval, 0.012-0.039). CONCLUSION: BD may be associated with a lower cancer-related morbidity compared with the general population of Korea.

Beh(c)et disease with venous sinus thrombosis / 中华风湿病学杂志

Objective To explore the key points of diagnosis and treatment of Behcet's disease with venous sinus thrombosis.Methods The diagnosis and treatment of a 25 year-old female patient with recurrent headaches was analyzed and discussed.Results Her headache was due to Behcet's disease with venous sinus thrombosis and her symptoms were gradually relieved.Conclusion Physician should be asked about the history in patients with persistent headache and the correct diagnosis can be made by MRI.

A Case of Vasculo-Behçet Disease Diagnosed by Right Atrial Mass and Inferior Vena Cava Thrombosis / 日本心臓血管外科学会雑誌

We report a rare case of 16-year-old boy who was given a diagnosis vasculo-Behçet disease after removing a right atrial thrombus. He was admitted to our hospital with abdominal pain and fever. He was underwent appendectomy for suspected appendicitis, but the appendix was normal. Additional image examinations revealed a mobile right atrial mass and inferior vena cava thrombosis, and the patient was sent to reoperation urgently to prevent pulmonary embolism. Surgery revealed the mass to be a thrombus. Vasculo-Behçet disease was diagnosed based on the patient's history and examination data. He was discharged on the 17th postoperative day. Cardiac mass excision should be immediately considered in such cases, and the differential diagnosis of Behçet disease was important for this case.

A Case of Vasculo-Behçet's Disease Complicated by an Intracardiac Thrombus / 日本心臓血管外科学会雑誌

We report a rare case of vasculo-Behçet's disease complicated by an intracardiac thrombus. A 48-year-old woman with a history of Behçet's disease and deep vein thrombosis in a lower limb, and who had been treated with colchicine and warfarin, was given a diagnosis of an appendectal tumor by colonoscopy. Preoperative examinations, including chest computed tomography, showed a pedunculated mobile tumor from her inferior <i>vena cava</i> (IVC) to the right atrium (RA). Urgent surgery was performed to prevent pulmonary embolism. We exposed the RA through a median sternotomy under cardiopulmonary bypass and extirpated the tumor that appeared macroscopically to be an organized thrombus attached to the IVC wall. The pathological diagnosis was organized thrombus. A month later, she underwent ileocecal resection and was given a pathological diagnosis of mucinous cystadenoma. Her postoperative course was uneventful. Intracardiac thrombus complicated by vasculo-Behçet's disease is rare, and it is important in the differential diagnosis of intracardiac tumor.

Surgical Repair of Various Pseudoaneurysms in 2 Patients with Vasculo-Behçet Disease / 日本心臓血管外科学会雑誌

Vasculo-Behçet disease (VBD) is a special type of Behçet disease (BD) involving some vascular disorders like aneurysmal formation, arterial occlusion, and venous thrombosis in various vessels. VBD has a poor prognosis due to aneurysmal rupture or recurrence of vascular disorders despite optimal treatment. However, definite diagnosis in BD is made on the basis of clinical features, and early diagnosis is difficult. We report 2 patients whose first clinical symptoms were femoral-pseudoaneurysms. They received a diagnosis of VBD after surgery. The first patient was a 69-year-old man, who underwent autologous-vein patch closure of a perforated region in the left femoral artery. One year later, he had a pseudoaneurysm of the right profunda femoris artery, which was ligated. The second patient was a 51-year-old man, who underwent the interposition of the saphenous vein for defective artery due to left superficial femoral-pseudoaneurysm.

A Case of Beh\ccet's Disease Successfully Treated with Keishibukuryogan, Whose Liver Damage Was Caused by Orengedokuto / 日本東洋医学雑誌

We present the case of 65 year-old male with Behçet's disease who was successfully treated with the Kampo medicine keishibukuryogan. The patient presented with hyperemic conjunctiva, ocular pain and uveitis at the age of 38, and was diagnosed with Behçet's disease. One year later he was affected with ulcerative glossitis, which was also diagnosed as a manifestation of Behçet's disease. At the age of 56 he was presented with acute arthritis of his ankle joint, followed by ulcerative stomatitis and glossitis. He was treated with colchicine and cyclophosphamide. Thereafter, he suffered from recurrent oral aphthous ulcers, and arthritis. Intractable oral ulcers, which did not respond to Western medicine, led him to seek Oriental herbal remedies. Keishibukuryogan was prescribed and, since then, his oral ulcers have completely subsided and his arthritis has not been a problem. He has been relieved from all manifestations of Behçet's disease.Thus, we believe that the Kampo medicine keishibukuryogan should be taken into consideration when treating Behçet's disease.Moreover, liver dysfunction caused by the Kampo medicine orengedokuto arose at an early stage of therapy, but immediately disappeared when its use was stopped. Pharmacoepidemiologically, Scutellariae radix, an ingredient of orengedokuto, was suspected of causing this liver dysfunction.

An increased expression of CD40/CD40L costimulatory molecules in erythema nodosum of patients with Beh(c)et's disease / 国际眼科杂志(Guoji Yanke Zazhi)

· AIM: To investigate the expression and the possible implication of CD40/CD40L costimulatory molecules in erythema nodosum of patients with Beh(c)et's disease.· METHODS: Sampling was done from erythema nodosum of 5 patients with Beh(c)et's disease and normal skin of 2 healthy individuals. Immunohistochemical staining was performed to examine the expression of CD4, CD8, CD19, CD68, HLA-DR,CD40 and CD40L molecules in the obtained tissues.· RESULTS: Approximately 90% of epidermic cells in erythema nodosum expressed CD40 molecule. In the dermis and subcutaneous tissue, a significantly increased number of CD4+Tcells, CD8+Tcells, CD19+cells, CD68+cells, HLA-DR+cells,CD40L+cells, and CD40+cells were observed in the erythema nodosum as compared with that in normal skin. Double staining showed that CD40L molecules were expressed on 45% of CD4+T cells. CD40 molecules were expressed on 100% CD68+ cells and 59.2% of HLA-DR+cells respectively.· CONCLUSION: A number of CD40/CD40L costimulatory molecules are upreguiated in the erythema nodosum of patients with Behcet's disease.

A Case of Brain Stem Neuro-Behcet's Disease Presenting as Paroxysmal Headache With Autonomic Features

Migraine without aura was significantly frequent in Beh et's disease patients. While, the relationship of TAC(trigeminal autonomic cephalalgia) and Beh et's disease was not known. And, it is thought that pathogenesis of proxysmal headache with autonomic features in neuroBehcet's disease as our case may be different from those of clust headache and TAC. A 45-year-old male admitted with left sided periobital poxysmal headache accompained by ipsilateral conjunctival injection and lacrimination with autonomic features. T2WMR image showed about 1.5x2.6x2.6 cm sized mass with high signal intensity surrounded by a rim of low signal intensity in pons. Enhanced MR image showed enhancing mass-like lesion inside the T2 high signal intensity. CSF study revealed neutrophilic pleocytosis with normal pressure, protein and glucose concentration. No malignant cell were seen. Antiphospholipid antibody and cryoglobuline was significantly increased. 3 days after hospitalvisit, oral ulcerations appeared in the patient. He was treated with high-dose steroid for a week and his headache and abnormal findings of CSF study became fully recovered. Two weeks later, the mass was nearly reduced in the pons on follow-up MR image. We suggest to include a careful interviewfor 2ndary TAC as the first neurologic symptom in the diagnostic work-up of Behet's disease.