Behcet Syndrome ; complications ; Hemorrhage ; etiology ; Humans ; Pharynx

Polychondritis is an inflammatory disorder that affects various catilagenous structures, and the clinical features include auricular, nasal and respiratory tract chondritis. It also involves the eyes, audiovestibular apparatus, joints and vascular structures. Polychondritis can be associated with several rheumatologic diseases such as systemic lupus erythematosus, rheumatoid arthritis and systemic vasculitis. However, polychondritis is a rare complication of Behcet's disease (BD) and only ten cases with combined BD and polychondritis have been reported on around the world. In this report, we describe a 40-year-old Korean man with BD who suffered from polychondritis that manifested as bilateral auricular chondritis, conjunctivitis and arthritis.
Polychondritis, Relapsing/*complications ; Male ; Humans ; Behcet Syndrome/*complications ; Adult

Behcet Syndrome ; complications ; Humans ; Male ; Middle Aged ; Myocardial Infarction ; complications

Behcet's syndrome is a multi-systemic and chronic disorder that affects many organs. It has been suggested that the diagnosis was based on the presence of the 'major' and 'minor' clinical criteria. When thromobophlebitis, arthritis, central nervous system or gastrointestinal lesions are also present. Behcet's syndrome will be thought to be present in the appropriate geographic area. We report a case of superior vena cava syndrome caused by Behcet's disease in a 40-year-old man with recurrent oral aphthous ulcers and skin rashes on the anterior chest wall. There were multiple thrombosis of the superior vena cava, innominate and subclavian veins. This patient also had a solitary cecal ulcer with an ileocecal fistula and downhill varix. The chest CT, veno-cavography, pulmonary angiography and colon study were taken and follow-up was performed.
Adult ; Behcet's Syndrome/diagnosis ; Behcet's Syndrome/complications* ; Cecal Diseases/complications ; Human ; Intestinal Fistula/complications ; Male ; Superior Vena Cava Syndrome/etiology* ; Superior Vena Cava Syndrome/diagnosis ; Ulcer/complications

Behcet's disease occurs with a high prevalence in the Far East including Korea. In this report we inspected 1,155 patients with Behcet's disease and collected information concerning the clinical and epidemiologic features of Behcet's disease in Korea. In summary, patients in their 30s were the most common, with the most common age of onset in the 20s; the sex ratio was 0.63:1 with female predominance; according to revised Shimizu's classification, the order of frequency of the different types was incomplete (38.2%), suspected (25.4%), possible (20.3%) and complete (16.1%); oral ulcers were the most frequent major symptom (97.5%) and the most common initial symptom (78.5%); erythema nodosum-like lesion (55.3%) was the most common type of skin lesion; a seasonal aggravation of the disease was observed in 21.3% of patients; and the most common type of childhood onset Behcet's disease was suspected type.
Adolescence ; Adult ; Age Distribution ; Aged ; Behcet's Syndrome/epidemiology* ; Behcet's Syndrome/complications ; Behcet's Syndrome/classification ; Child ; Female ; Human ; Korea/epidemiology ; Male ; Middle Age ; Sex Distribution

Adult ; Behcet Syndrome ; complications ; Female ; Heart Atria ; pathology ; Humans ; Myocardial Infarction ; complications ; Pericardial Effusion ; complications

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.
Adult ; Animals ; Behcet Syndrome/complications* ; Guillain-Barre Syndrome ; Humans ; Immunoglobulins, Intravenous ; Male ; Methylprednisolone ; Quality of Life ; Sheep

Malignant lymphoma complicated by Behcet's disease (BD) is a rare clinical entity. We report a case of BD complicated by malignant lymphoma in a 26-year-old male patient. The patient was diagnosed to have terminal ileum extranodal NK/T-cell lymphoma (nasal type) during treatment for BD with cyclophosphamide (CTX), immunoregulants and biological agents. This is the first case reported in China and the second case globally. The pathogenesis of BD complicated by malignant lymphoma remains unclear. We reviewed the relevant literatures to summarize the clinical characteristics of BD complicated by extranodal NK/T-cell lymphoma (nasal type) and discuss the possible pathogenesis in light of immunology, EB virus infection and medications.
Adult ; Behcet Syndrome ; complications ; Humans ; Lymphoma, Extranodal NK-T-Cell ; complications ; Male

Adolescent ; Arterial Occlusive Diseases ; complications ; Behcet Syndrome ; complications ; Humans ; Hypertension ; etiology ; Hypertensive Encephalopathy ; etiology ; Male

The clinical features of cutaneous manifestations in 411 patients with Behcet's syndrome were studied. 302 patients (73.5%) had skin lesions. The frequency with which the following skin lesions were noted was, in decreasing order; erythema nodosum-like lesion, papulopustular eruption, erythema multiforme-like lesion, thrombophlebitis, ulcer and Sweet's syndrome-like lesion. More than two types of skin lesions were seen in 86 patients (28.5%), the combination of the skin lesions being in decreasing order, erythema nodosum-like lesion and papulopustular eruption; erythema multiforme-like lesion and papulopustular lesion; erythema nodosum-like lesion and thrombophlebitis. A skin pathergy test was performed on 245 patients, and a positive reaction was seen in 97 patients (39.6%). This study showed the high incidence, wide spectrum and importance of skin lesions as a major symptom in Behcet's syndrome.
Adolescent ; Adult ; Aged ; Behcet Syndrome/complications* ; Behcet Syndrome/epidemiology ; Child ; Female ; Human ; Male ; Middle Age ; Skin Diseases/classification ; Skin Diseases/epidemiology* ; Skin Diseases/etiology