Genital Ulcers as an Unusual Sign of Periodic Fever,Aphthous Stomatitis,Pharyngotonsillitis, Cervical Adenopathy Syndrome: A Novel Symptom?

Abstract: Periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy (PFAPA) syndrome, which is characterized by periodic episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis, is of unknown etiology and manifests usually before 5 years of age. A patient with periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome simultaneously presenting with genital ulcers has not been reported previously. We describe a 12‐year‐old Chinese girl with a 2‐year history of periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome who exhibited vulvar ulcers accompanying an episode of febrile periodic fever, aphthous stomatitis, pharyngotonsillitis, and cervical adenopathy. Although during a 1‐year follow‐up this girl did not manifest typical symptoms/signs of Behçet’s disease except recurrent oral aphthae and genital ulcers, it is possible that periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome and Behçet’s disease could have overlapping manifestations. Furthermore, this report would add to the evidence of a wide variation in the clinical symptomatology of PFAPA syndrome.     

Vitiligo Koebnerized by Behçet Disease Genital Ulceration

A 50-year-old man was diagnosed with Behçet disease (BD) on the basis of an 8-year history of episodes of recurrent oral aphthous ulcers, papulopustular lesions on physical examination, and a positive pathergy test. Two years prior to diagnosis of BD, vitiligo had also been diagnosed on the basis of hypopigmented lesions on the scrotum following genital ulceration. To our knowledge, this is the first case of incidental coexistence of BD and vitiligo, and, furthermore, the first case of koebnerization of genital ulceration of BD.     

Behçet syndrome: A great imitator

Behçet syndrome is considered to be a multisystemic vasculitis involving the skin, mucosa, eyes, joints, nervous system, cardiovascular system, and gastrointestinal system. The exact pathogenesis of the disease is unknown, but autoimmune factors are thought to play the main role. Vasculitis in Behçet syndrome can involve any kind and size of vessels, and this explains why the disease has the ability of multisystemic involvement. The commonest clinical presentation of Behçet syndrome is recurrent and painful mucocutaneous ulcerations known as aphthosis. The other clinical manifestations vary among patients and populations. The disease tends to be more severe in men. Ocular, vascular, and central nervous system involvements are the major causes of morbidity and mortality. Behçet syndrome is a mimicker of many diseases with its several faces and considered as one of the great imitators in dermatology.     

A case of pediatric Behçet's disease with intestinal involvement

We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behçet's disease with intestinal involvement. According to the literature, pediatric Behçet's disease is characterizesd by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behçet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behçet's disease complain of abdominal pain     

Behçet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease

A 47‐year‐old woman presented with a 2‐month history of generalized arthralgia and a 10‐day history of oral aphthous ulcers. After hospitalization, papulopustular lesions and perianal ulcerations developed. Pathergy test was positive and ophthalmological examination was normal. The presence of oral aphthous ulcers, genital ulcerations, papulopustular lesions and arthralgia, and the positive pathergy test suggested the diagnosis of Behçet's disease (BD). In a few days, positive pathergy reactions and papulopustular lesions evolved into bullous lesions, which were diagnosed dermatopathologically as pyoderma gangrenosum. Two days after the presentation of papulopustular lesions, the patient experienced diarrhoea accompanied by bloody stools and mucus. Histopathological examination of biopsy specimens showed no vasculitis but revealed findings suggestive of Crohn's disease. The patient responded well to treatment with systemic steroids and 5‐aminosalicylic acid. Our case demonstrates that the differential diagnosis of BD and inflammatory bowel disease may be perplexing and that these two diseases may be closely related.     

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms

Entero‐Behçet's disease coexisting with long‐term epilepsy and schizophrenia‐like symptoms is presented. A 43‐year‐old woman presented with repeatedly occurring aphthous stomatitis for several years. She had been treated for absence seizures, epilepsy and schizophrenia since she was 9 years old. She presented with multiple aphthous stomatitis on her gingiva, erythema nodosum‐like symptoms on the right lateral aspect of her leg and genital ulcers on her perianal area. She also showed polyarthritis. Laboratory examinations revealed elevated C‐reactive protein, elevated neutrophil counts, decreased serum Fe and elevated serum Cu. Histological examination showed perivascular neutrophil and mononuclear cell infiltrates and eosinophilic change of the vessel wall in the lobules of subcutaneous fat tissue. Six weeks after the oral prednisolone therapy, she showed resolution of aphthous stomatitis, folliculitis‐like eruption and genital ulcer. She experienced severe abdominal pain after the start of treatment of Behçet's disease. Plain computed tomography revealed edematous change in the appendix, and ascending and transverse colon. These results led to the diagnosis of entero‐Behçet's disease acute exaggeration. Treatment with infliximab (300 mg/once) was started. Eight weeks after the start of infliximab, her abdominal pain disappeared and C‐reactive protein decreased, followed by the successful change to adalimumab infusion therapy.     

Fever,Irritability, and Ulcerations in an Infant

Abstract: A young infant with fever and oral ulcerations is presented herein. Extensive infectious, nutritional, immunodeficiency and autoimmune work up was performed. Pertinent maternal history suggested the potential diagnosis of neonatal Behçet’s, and treatment for this condition resulted in rapid and sustained clinical improvement.     

Prognosis and Clinical Relevance of Recurrent Oral Ulceration in Behçet's Disease

There is no way of predicting whether a patient with recurrent oral ulcerations (ROU) will develop Behçet's disease (BD). In the absence of a valid laboratory test to exclude BD, such oral ulcerations result in a diagnostic problem when they occur as the sole and earliest manifestation of disease. We assessed the prognosis of ROU by performing prospective evaluations of 67 patients who had only a history of ROU and were registered at the Behçet's Disease Specialty Clinic at Severance Hospital of Yonsei University, Seoul, Korea. Thirty-five patients (52.2%) developed overt manifestations of BD at an average of 7.7 years after the onset of ROU. The frequency of recurrence was 9.8 times per year in progressive cases. From these results, it appears that highly recurrent ROU is a warning signal for BD. Careful examinations of patients, including their minor symptoms, additional laboratory tests, and regular follow-ups by physicians are required for proper diagnosis.     

Treatment of Behçet Disease with Indomethacin

ABSTRACT: Thirty patients with Behçet disease (BD) were treated with oral indomethacin 25 mg four times daily for 3 months as an open label study. The study population consisted of 13 women and 17 men from 15 to 45 years of age (mean 27 years). At the initiation of the therapy, 28 patients had oral aphthous lesions, 23 had joint involvement, 13 had genital ulcerations, 8 had cutaneous lesions, and 4 had eye involvement. Eighty-eight percent of patients with skin lesions, 80% with joint involvement, 43% with oral aphthous lesions, and 38% with genital ulcerations responded to indomethacin therapy. In three patients indomethacin was changed to the suppository form due to gastrointestinal side effects. Although it is difficult to assess the efficacy of indomethacin in BD because of the intermittent nature of symptoms, the majority of patients showed improvement in their symptoms of arthritis and skin lesions. This study indicates that indomethacin can be used as effective therapy particularly in patients with joint involvement.     

Association of class I HLA antigens with the clinical manifestations of Turkish patients with Behçet's disease

Summary Genetic factors appear to be important in the pathogenesis of Behçet's disease. Although it is known to be strongly associated with HLA‐B51, the association of HLA class I antigens with specific clinical findings of the disease has not been studied extensively and the few studies are conflicting. The aim of this study was to investigate the association of HLA class I alleles with the manifestations of Behçet's disease in Turkish patients. Eighty‐five patients with Behçet's disease were typed for HLA‐A, B, and C antigens with the serologic, standard microlymphocytotoxicity technique. Possible associations of the HLA complex with clinical findings of Behçet's disease were examined. Statistically significant findings are as follows (P < 0.05): increased HLA‐B51 and decreased HLA‐B35 frequency in patients with thrombophlebitis, increased HLA‐A29 and decreased HLA‐Bw6 frequency in patients with ocular involvement, decreased HLA‐Cw2 frequency in patients with erythema nodosum, and decreased HLA‐Cw7 frequency in patients with genital ulceration. Of particular note, the results of this study suggest that the presence of HLA‐B51 and the absence of HLA‐B35 can be regarded as laboratory risk factors of venous thrombosis in patients with Behçet's disease.     

Use of Colchicine in the Treatment of Behçet's Disease

ABSTRACT: To date, various treatment modalities have failed to produce consistent improvement in patients having Behçet's disease. Recent reports in the literature, however, have shown promising results with the use of colchicine. Our patient, an 18-year-old white woman, satisfied the 1977 Istanbul Conference criteria for Behçet's disease by exhibiting ocular inflammation and a five-year history of severe, recurrent oral and vaginal ulcerations. She was treated with 0.6 mg of colchicine orally twice daily, and at the end of two weeks of treatment, the oral lesions had completely resolved, and the vaginal ulcers were reduced in diameter and depth to at least one half. Within one month, all oral and Vaginal ulcers were completely healed.     

BEHÇET'S DISEASE: CLINICAL STUDY OF JORDANIAN PATIENTS

Background. Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by the triad of oral and genital ulcers and ocular lesions. The etiology is unknown. This is the first report giving details of the clinical manifestations of Behçet's disease in Jordanian patients. Methods. Twenty patients with Behçet's disease were studied to determine the clinical pattern in the North of Jordan. The patients were seen in Princess Basma Teaching Hospital in North Jordan. They presented to various clinics and underwent full clinical examination. Data for each patient on all features of Behçet's disease were recorded on a standard form. A comparison was made between Behçet's disease in Jordan and other countries in the region. Results. Of the 20 patients, 14 were men and six women, giving a ratio of 2.3:1. Their ages ranged from 14 to 58 years. All had mouth ulcers, 65% genital ulcers, 65% ocular involvement, 55% joint involvement, 35% skin lesions, 20% vascular lesions, and 5% gastrointestinal involvement. The oral ulcers were the first manifestation of the disease process in 70% of our patients. Skin lesions, genital ulcers, and involvement of the central nervous system and the pulmonary system were less frequent in our study. Conclusions. Although it is difficult to obtain figures as to the incidence and prevalence of Behçet's disease in Jordan, the clinical manifestations, with a few exceptions, are similar to those in other countries in the region.     

Sweet's syndrome associated with Behçet's disease

A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with Behçet's disease occurring in a 48 year old woman is reported. She fulfilled the diagnostic criteria for both Sweet's syndrome and Behçet's disease. There have been several reports of this association. Herpes simplex virus was isolated from a genital ulcer in our patient. The possible role of herpes simplex virus in this interesting association of Behçet's disease and Sweet's syndrome is discussed and a review of the literature is made.     

Behçet’s disease and vitiligo in two brothers: coincidence or association?

Behçet’s disease (BD) and vitiligo are disorders with unknown aetiology. We report on two brothers with ocular Behcet’s disease who had advanced unilateral ophthalmic symptoms associated with vitiligo. The two brothers had recurrent oral and genital ulceration, uveitis and white patches on their skin. The most probable hypothesis for the aetiology of BD is that of an autoimmune reaction in genetically predisposed individuals, with vasculitis as the main pathological hallmark. Despite many years of research, the specific causes of vitiligo remain obscure, and the most advanced aetiological hypothesis remains that of autoimmunity. To our knowledge, this is the first reported case within the literature of BD associated with vitiligo. The existence of the two different disorders is noteworthy as they were observed in two brothers during the same period in their lives, with very similar clinical observations.     

Development of Behçet's disease in a Caucasian with human leukocyte antigen B51 after immigration to Japan

We herein report a 32‐year‐old Caucasian woman with human leukocyte antigen (HLA)‐B51 who developed Behçet’s disease after immigration to Japan. She was born and raised in Finland and had never experienced any clinical symptoms related to Behçet’s disease before immigration to Japan. None of her family members in Finland had similar problems. Two years after the immigration, she developed typical clinical symptoms of Behçet’s disease. This report strongly supports the canonical notion that the interaction of genetic factors, such as HLA‐B51, with environmental factors specific to the countries in the Silk Road area, including Japan, may play a pivotal role in the pathogenesis of this disorder.     

Cardiac findings in Behçet’s patients

Behçet’s disease is a chronic multi‐system inflammatory disorder and the severity and clinical manifestations of Behçet’s patients may show geographic variation. We aimed to detect the cardiac findings in 30 Behçet’s patients and compare them with the normal population (n = 29). We used color‐doppler echocardiography and transesophageal echocardiography in combination. We calculated manually QT intervals and QT dispersion (QTd) from twelve‐lead ECG recordings. There was no E/A inversion and coronary ischemia in all patients or control group. The E velocity difference between groups was not significant. The mean A velocity was significantly lower in Behçet’s patients than normal group. The mean DT was 154.4 ± 5.8 msec in Behçet’s patients and 122.59 ± 0.96 msec in control group (P < 0.0001). The mean IVRT was 75.66 ± 1.36 msec in Behçet’s patients and 69.1 ± 0.55 msec in control group (P < 0.0001). There was no QTc time difference between the Behçet’s patients and the control group. The mean QT dispersion (QTd) interval was 45.46 ± 2.65 msec in Behçet’s patients and 31.83 ± 1.23 msec in control group (P < 0.0001). Atrial septal aneurysm, mitral valve prolapse and insufficiency, tricuspid valve insufficieny, and pulmonary hypertension frequencies in Behçet’s patients were significantly higher than in the control group. We concluded that Behçet’s cardiac involvement may effect cardiac structure and cause diastolic dysfunction, electrical instability and structural abnormalities. We also concluded that cardiac involvement in Behçet’s disease may be specific for this geographic area.     

Granulysin-producing cytotoxic T cells in the mucocutaneous lesions of Behçet disease: a distinct inflammatory response from erythema nodosum

Background. Cytotoxic T lymphocytes (CTLs) have been recognized as an important effector cell in Behçet disease (BD). Granulysin is a cytolytic granule protein expressed by CTLs and natural killer cells. Aim. To evaluate the involvement of granulysin‐producing T cells in the pathogenesis of BD. Methods. Using immunohistochemistry, lymphocyte subsets expressing granulysin were investigated in mucocutaneous lesions of BD. Serum granulysin levels were assayed by ELISA. Results. Granulysin‐positive cells were seen in specimens from oral ulcers, genital ulcers and acne‐like eruptions, but not erythema nodosum‐like lesions. Both CD4+ and CD8+ T cells expressed granulysin. Serum granulysin levels did not correlate with disease activity in BD. Conclusion. Immune reactions mediated by granulysin‐positive CTLs may play an important role in the pathogenesis of acne‐like eruptions, oral ulcers and genital ulcers in BD.     

Dapsone in Behçet's disease: a double-blind,placebo-controlled,cross-over study

The study was designed to investigate the effects of dapsone in the treatment of mucocutaneous manifestations of Behçet's disease and the possible prophylactic role of dapsone in a double blind/placebo controlled clinical trial. Twenty patients diagnosed according to the International Study Group criteria as Behçet's disease were included in the study. Patients were randomly allocated to receive either dapsone 100 mg daily or placebo for three months in a double‐blind manner. After three months, patients were crossed over and followed for a further three months. Patients were followed up in each visit by assessing the number, size, duration and frequency of oral and genital ulcers, other cutaneous manifestations, and systemic manifestations of the disease. A pathergy test was done on each visit. Laboratory investigations included hemoglobin concentration, white blood cell count, ESR, and C‐reactive protein. In dapsone‐treated patients, there were significant reductions in the oral and genital ulcer parameters as well as the incidence of other cutanous and systemic manifestations. In the placebo‐treated group, there were no significant changes in these parameters. The pathergy test result as well as those of other laboratory tests were all decreased in the dapsone‐treated group. Although this study was a small scale study, it shows that dapsone was effective in treatment of mucocutaneous manifestations of Behçet's disease and possibly in prophylaxis against systemic manifestations of the disease. This result should lead to a larger scale study with a longer duration of follow‐up.     

Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease

Background Behçet's disease is a multisystem disease featuring mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement and occurs with a high prevalence in the Mediterranean including Turkey. Higher incidence of severe clinical course and systemic involvement is observed in male patients. Objective To determine the influence of sex on the clinical course of Behçet’ s disease. Methods We retrospectively evaluated the clinical findings of 2313 Behçet patients followed up at the multidisciplinary Behçet's Disease Center at Ankara University. Results The male/female patient ratio was 1.03. Oral aphthae was seen in all patients. In male Behçet patients, the prevalence of mucocutaneous lesions and systemic manifestations was as follows: 85.6% genital aphthae, 45.5% erythema nodosum, 59.5% papulopustular lesions, 17.5% thrombophlebitis, 38.1% ocular involvement, 11.3% articular involvement, 11.7% vascular involvement, 3.3% neurologic involvement, 1.4% gastrointestinal involvement, and 1.8% pulmonary involvement. In female Behçet patients, the prevalence of manifestations were as follows: 91% genital aphthae, 49.8% erythema nodosum, 48.3% papulopustular lesions, 3.5% thrombophlebitis, 19.8% ocular involvement, 11.8% articular involvement, 2.1% vascular involvement, 1.3% neurologic involvement, 1.4% gastrointestinal involvement, and 0.03% pulmonary involvement. Conclusions Only genital aphthae and erythema nodosum were more frequent in females. On the other hand papulopustular eruptions, thrombophlebitis, ocular, neurologic, pulmonary and vascular involvement were more frequent in males. While female patients had the best prognosis, male patients had a worse overall prognosis than females.     

Formation of arcuate crusted lesions after non-ablative 1450-nm diode laser treatment equipped with a dynamic cooling device

Abstract

Behçet's disease (BD) is a chronic systemic inflammatory disorder of unknown etiology with variable clinical manifestations. HLA-B51 allele is the most strongly associated known genetic factor. The mucocutaneous lesions (oral aphthae, genital aphthae, skin lesions such as pseudofolliculitis) constitute the hallmark of the disease, but also gastrointestinal, vascular, central nervous systems, and others may be involved. We report a case of a young man affected with Behçet's disease who presented facial telangiectasias and striae rubra in the inner region of his arms and at the level of his hips, as uncommon minor superficial vascular manifestations of BD. To manage them we have subjected the patient to a cycle of Intense Pulsed Light (IPL) therapy. Our findings showed that the use of IPL is a safe and effective treatment for telangiectasias and striae rubra, also in the complex clinical condition of Behçet's disease. In fact, the treatments were well tolerated, no sign of scarring or hyper/hypopigmentation was reported and we obtained a significant improvement of the lesions in terms of color and size of them.