Sweet's syndrome in Behçet's disease

Few cases of Sweet's syndrome have been reported in patients with Behçet's disease.Case reportA 47-year-old woman with five year history of oral and genital ulcers that had not been investigated previously was admitted for an eruption of tender, erythematous, vesicle-like papules over the face, neck, palms, and legs. She reported polyarthralgia and weight loss of 6 kg over the previous month. At admission, she had a fever of 38.5 °C and conjunctivitis. Joint tenderness to mobilization without evidence of synovitis was noted. Laboratory tests showed inflammation and leukocytosis (12.5 × 10⁹/L) with 74% neutrophils. The skin biopsy was typical for Sweet's syndrome, and the pathergy test was positive. Glucocorticoid therapy 40 mg/day and colchicine were effective in alleviating the manifestations.DiscussionOverlap exists between the clinical manifestations of Sweet's syndrome and Behçet's disease. Data from the literature suggest that Behçet's disease may be among the conditions that underlie Sweet's syndrome.     

Late onset Behçet's disease

Beh?et's disease is characterized by recurrent mouth ulcerations associated with two of the following clinical manifestations: recurrent genital ulcerations, ophthalmic and cutaneous lesions and a positive Pathergy test. The young adult is the most affected, usually in the third and fourth decades, with an exceptional onset after the age of 60 years. We report two cass of late onset Beh?et's disease, occurring after the age of 70 years. The first case is a 70-year-old Lebanese male complaining of asymmetrical oligoarthritis, uveitis, oral ulcerations, ear chondritis, pseudofolliculitis and pustular rash, all corticosensitive. The second is the case of a 72-year-old Lebanese female who presented with posterior uveitis and retinal vasculitis, bipolar cutaneous ulcerations and a positive family history of Beh?et's disease; all the symptoms were stabilized by immunosuppressive therapy. We discuss the differential diagnoses and the particularities of this late onset and emphasize the necessity of establishing an early diagnosis of Beh?et's disease, even at an older age, and the need to start an adequate treatment in order to prevent serious ophtalmic and systemic complications.     

Recurrent epididymo-orchitis in patients with Behçet's disease

PURPOSE: We study the clinical features of epididymo-orchitis in patients with Beh?et's disease. MATERIALS AND METHODS: Of 57 men with Beh?et's disease 7 had epididymo-orchitis during the course of disease. A questionnaire was applied to all cases and the clinical features were recorded. HLA class 1 was determined and a pathergy test was performed. RESULTS: All 7 patients fulfilled the international study group criteria for Beh?et's disease. All patients had recurrent oral ulcers, skin lesions (folliculitis, erythema nodosum-like lesions) and uveitis, and 6 had recurrent genital ulcers, 2 arthritis, 1 vasculitis and 4 central nervous system involvement. The pathergy test was positive in 4 patients and all had B5(51). The allele B5101 was present in 6 patients and 1 had B5102. Recurrences were observed in 5 patients. Concurrent symptoms with epididymo-orchitis consisted of oral ulcers, fever, uveitis, arthritis and penile ulcer. Colchicine, glucocorticoids, nonsteroidal anti-inflammatory drugs and cyclosporine were administered. CONCLUSIONS: Epididymo-orchitis in patients with Beh?et's disease is not as infrequent as believed. It was not the first manifestation of the disease but developed during followup. In any patient with epididymo-orchitis Beh?et's disease should be considered, particularly in regions with a high prevalence of the disease.     

Behçet's disease: a case report

While Beh?et's disease is an uncommon presentation to a podiatric clinical setting, it is nonetheless a fascinating and poorly understood disease as demonstrated by the current and historical research available about the topic. Much debate remains about the disease cause, course and treatment goals. Only through further research into the specific components of this multisystem disease will medicine be more fully able to address the needs of the patient. This discussion should offer the podiatric physician an overview of the disease as well as available treatment options.     

Vascular involvement in Behçet's disease. Two case reports

Arterial involvement is rare in Beh?et's disease but can be at the forefront of the clinical picture and cause life-threatening complications. CASE REPORTS: A 36-year-old man had Beh?et's disease with an aortographically documented aneurysm of the abdominal aorta as the inaugural manifestation. He had oral and genital ulcers. Funduscopy showed periphlebitis. In a 38-year-old man with an 8-year history of Beh?et's disease, pulmonary and coronary artery aneurysms developed, as well as intracardiac and venous thromboses. DISCUSSION: Arterial involvement occurs in 3-5% of patients with Beh?et's disease and usually manifests as multiple spindle-shaped aneurysms. Intracardiac thrombosis and cardiac aneurysm are exceedingly rare. Our patient had an extremely unusual presentation given the low rate of occurrence of arterial lesions in Beh?et's disease. CONCLUSION: Arterial involvement in Beh?et's disease raises treatment challenges because the lesions tend to recur and can cause life-threatening complications.     

Coronary artery disease and coronary artery bypass grafting in Behçet's disease

Abstract There is a high frequency of pseudoaneurysm formation in patients with Behçet's disease and their inflammed and fragile tissues are difficult to manipulate. Five patients with Behçet's disease were referred to our cardiovascular surgery department for coronary artery bypass grafting (CABG). Three of them were operated and two were treated medically. Patients that were managed medically had left anterior descending (LAD) lesions below 80% and their stable angina pectoris responded well to medication. There was no early mortality and morbidity. One patient developed pseudoaneurysm of ascending aorta and femoral artery. This patient died in the late postoperative period. At follow‐up the operated patients were in Canadian Cardiovascular Society (CCS) Class I, while the medically treated patients were in CCS Class II. Mean follow‐up period was 41 ± 36.21 months. Coronary artery disease (CAD) is extremely rare detected in patients with Behçet's disease. The affected patients are usually young males. Coronary artery bypass grafting is also rarely performed in these patients and long‐term results of such operations are not available in the literature. We present five patients with Behçet's disease that had CAD, three operated and two medically treated, and report their long‐term results. We suggest a conservative approach in patients with Behçet's disease because of the high risk of pseudoaneurysm formation in the postoperative period. If CABG cannot be avoided we recommend operating the patients on the beating heart with minimal aortic manipulation.     

Behçet's Disease

Clinical and imaging data suggest that Beh?et's disease (BD) can present with a variety of neurologic complications, which may be subclassified into two forms. One is attributable to small venous inflammatory disease with focal or multifocal central nervous system involvement and is seen in the majority of patients. It is designated Central Nervous System-Neuro-Beh?et syndrome (CNS-NBS). The other form is due to cerebral venous sinus thrombosis and has limited symptoms and a better prognosis. It is very uncommon for these two types of involvement to occur in the same individual. It is very likely that the two major forms have different pathogeneses. Patients with small vein inflammation should be considered for aggressive treatment. A substantial number of patients in this group will have a relapsing-remitting course; for some, this will evolve into a secondary progressive course. A few patients from this group will have progressive CNS dysfunction from the onset. Acute attacks of CNS-NBS are treated with either oral prednisone (1 mg/kg/d up to 4 weeks or until improvement is observed) or high-dose intravenous methylprednisolone (IVMP), 1 g/d for 5 to 7 days. After either treatment, an oral tapering dose of glucocorticoids should be given over 2 to 3 months to avoid early relapses. Some patients may require the long-term use of low maintenance doses of glucocorticoids to prevent exacerbations. Immunosuppressive agents (eg, azathioprine, cyclosporine A, cyclophosphamide, and chlorambucil), given either alone or in different combinations (eg, azathioprine with cyclosporine) for the long-term treatment of various systemic manifestations of BD, have not been shown to prevent the development of the neurologic complications of the disease, reduce its exacerbations, or stop its progression. Immunomodulatory treatments such as interferon alfa and thalidomide have been shown to be effective in treating some of the systemic manifestations of BD, but there is no information on their effects on the development and progression of CNS-NBS. Cerebral venous sinus thrombosis in BD is treated with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin given together with a short course of glucocorticoids. Evidence of benefit from this treatment, however, is weak.     

Bladder involvement in Behçet's syndrome

PURPOSE: We report our clinical experience with Beh?et's syndrome and bladder involvement. MATERIALS AND METHODS: From April 1991 through July 1996, 7 men and 1 woman 25 to 53 years old with Beh?et's syndrome were evaluated for lower urinary tract symptoms (7) or hematuria (1). Of 8 patients 5 had neurological involvement. Evaluation consisted of history, physical examination, urinalysis and urine culture, excretory urography, urodynamic studies, urethrocystoscopy, bladder biopsies and histopathological examination. RESULTS: Cystoscopy revealed bladder ulcer in 1 patient and an indurated, hypervascular lesion in another with bilateral hydronephrosis. The most common urodynamic finding was detrusor overactivity. Of 4 patients with poor compliance 1 had additional sphincteric deficiency. Common histopathological features were moderate and marked thickening of bladder vessel walls. Lymphocytic vascular reaction was present in 2 patients and lymphocytic vasculitis in 1. Clamshell augmentation ileocystoplasty was performed in 3 patients, including 1 who also underwent a sphincter enhancement procedure. The remaining 5 patients received various nonsurgical treatment. CONCLUSIONS: Various types of voiding dysfunction relating to bladder and sphincteric components in both phases of micturition can be seen in Beh?et's syndrome. Voiding dysfunction can be due to either neurological or direct bladder involvement. Augmentation ileocystoplasty is a good treatment option for Beh?et's syndrome with severe bladder involvement.     

Giant coronary artery aneurysm in a patient with Behçet's disease

Abstract Behçet's disease is a rare autoimmune vasculitis that may cause coronary artery aneurysms. We discuss the evaluation and management decisions for a 19‐year‐old female with a giant rapidly expanding aneurysm of the proximal left anterior descending coronary artery and Behçet's disease. (J Card Surg 2011;26:268‐270)     

Bilateral nipple necrosis in a breastfeeding woman with Behçet's disease

Beh?et's disease is a chronic, multisystem disorder. A 23-year-old female patient diagnosed with Beh?et's disease 3 years earlier was admitted with deep ulcers on both areolae caused by detachment of the nipples following breastfeeding since spontaneous delivery of her baby. Physical examination revealed a necrotic ulcer crater 2 cm in diameter on each breast, with the lactiferous ducts draining into these. Incisional biopsy sample from the areola ulcer border on the left breast showed leukocytoclastic vasculitis. Breastfeeding was stopped, and local wound care and systemic corticosteroid therapy were started. After 4 weeks, epithelialization tissue filled the ulcers, leveling the crater base with the areola. Bilateral nipple necrosis in a breastfeeding woman is a rare entity. It is thought that in the present case minor trauma caused by breastfeeding accompanied by neutrophilic infiltration resulting from amplified inflammatory response and leukocytoclastic vasculitis attributable to Beh?et's disease was responsible for the necrosis.     

Vascular complications after surgical repair of aneurysms in Behçet's disease

The poor prognosis of vasculo Behcet's disease is often due to postoperative vascular complications (false aneurysm and graft occlusion). We report a case of an abdominal aortic aneurysm associated with an aneurysm of the left common femoral artery in a 23-year-old Portuguese man. The primary treatment was surgical (aneurysmectomy and prosthetic revascularization). The early occlusion of the left femoral revascularized artery was treated surgically with a new bypass. The occlusion of the right limb of the aortoiliac graft was asymptomatic and was not treated. Two months after admission to our hospital, the stenosis of the infrarenal aorta successfully treated by angioplasty via the occluded right limb of the graft. The patient was followed up for 18 months. He could only walk a short distance and had rest pain in the left foot. Magnetic resonance angiography showed a false aneurysm of the infrarenal aorta, and an occlusion of the remaining left limb of the aortoiliac graft. The endovascular treatment performed does not avoid the need for surgical treatment, because occlusion and false aneurysm may occur after dilatation. The endovascular approach can also be used during a sudden inflammatory surge, and makes it possible to wait for a quiescent period when surgery can be performed.