Behçet syndrome

We present a 34-year-old man with a two-year history of aphthous stomatitis, who later developed painful, erythematous nodules on his lower extremities. A pathergy test was positive, and the diagnosis of Beh?et syndrome (BS) was made. It is important for the dermatologist to recognize the wide variety of cutaneous manifestations of this disorder. A pathergy test is a simple diagnostic tool that may assist in making a diagnosis. Case reports of other unusual skin manifestations in BS also are reviewed.     

Behçet disease

Beh?et's disease is a multisystem inflammatory disorder of unknown origin, characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis, central nervous system, and vascular disease. There is no pathognomonic laboratory test, but there are clinical criteria to assist in establishing the diagnosis. Beh?et's is most common along the Silk Road. It is particularly common among persons who have the HLA-B51 major histocompatibility type. Cutaneous lesions include pustules, erythema nodosum-like lesions, Sweet's-like lesions, pyoderma gangrenosum-ike lesions, and pathergy. The major cutaneous findings may be classified as neutrophilic vascular reactions. There is considerable morbidity resulting from Beh?et's disease, most notably a high risk of blindness from ocular involvement. Mortality may occur as a result of neurologic or vascular disease or gastrointestinal perforation.     

Patient Characteristics in Behçet Disease

Background: Behçet disease (BD) is a chronic, inflammatory, multisystemic vasculitic disorder with a wide spectrum of clinical presentations. The highest prevalence is seen in Turkey. Specific diagnostic tools are yet to be discovered; thus, the diagnosis relies on physicians being acquainted with the symptoms and signs of the disease. Objective: To investigate the epidemiologic characteristics of BD and to emphasize the typical clinical and laboratory characteristics. Methods: This was a retrospective analysis of all the BD patients attending the Ankara Numune Education and Research Hospital throughout the years 2001–4. Diagnosis of BD was made according to the International Study Group criteria. A total of 213 patients were evaluated with respect to family history, clinical features, pathergy test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), antistreptolysin O, and ferritin levels. When assessing disease activity, the active clinical manifestations on the day of the examination were taken into account. Correlations were analyzed between sex and age distribution, age of onset, disease duration, and family history; and between family history and age of onset, pathergy, clinical manifestations, and laboratory parameters. Correlations were also evaluated between pathergy positivity and clinical manifestations, and laboratory parameters. Correlations between activity scores and age of onset, duration, sex, family history, and laboratory data were also analyzed. Results: The female : male ratio was 1.04, and the mean age of onset was 27 years. Family history did not affect age of onset or disease severity. Men presented with more active disease, and there was a weak but positive correlation between disease activity and CRP. No correlation was observed between duration and age of disease onset. The most common clinical presentations were oral aphthous lesions, genital ulcers, and skin lesions. Men more commonly presented with papulopustular lesions, pathergy positivity, and vascular, eye, and renal involvement, and women presented with arthritis/arthralgia more commonly than men. Vascular lesions, ESR, and CRP showed significant relationships with pathergy reaction. Eye involvement was not affected by age of onset. Conclusions: We believe our results indicate that the pathogenesis of BD is multifactorial. Hormonal factors seem to be of some influence, while genetic background and environmental factors seem to be the major contributors. Infections seem to be among the triggering environmental factors. Predisposing genes may affect the influence of environmental factors. Prevalence studies should be carried out periodically in those countries with a high prevalence ofBDto keep up with the changing dynamics of the disease, which may also shed light on the as-yet unknown pathogenesis of BD.     

A famous Turkish dermatologist, Dr. Hulusi Behçet

Dr. Hulusi Beh?et (1889-1948) is a famous Turkish dermatologist. He was born in Istanbul on February 20, 1889. His father was Ahmet Beh?et and his mother Ayqse Beh?et was also Ahmet's cousin. After the Turkish Republic was established and the "Family Name Law" was accepted, his father Ahmet Beh?et, who was among the friends of Mustafa Kemal Atatürk, the founder of Turkish Republic, received private permission to use his father's name Beh?et. Dr. Hulusi Beh?et pursued his education at Gülhane Military Medical Academy. After he had become a medical doctor, he specialized in dermatology and venereal disease at Gülhane Military Medical Academy and he completed his specialization in 1914. His first observations on Beh?et's Disease started with a patient he met between 1924-1925. Dr. Beh?et followed the symptoms of three patients whom he had had for years, then he decided that they were the symptoms of a new disease (1936). He published these cases in the Archives of Dermatology and Veneral Disease. He died from a sudden heart attack on March 8, 1948. Today, this disease is universally called Beh?et's Disease in medical literature.     

Prevalence of Metabolic Syndrome in Behçet Disease: A Case-Control Study in Turkey

Background

Chronic inflammatory diseases such as psoriasis, rheumatoid arthritis, and inflammatory bowel diseases have been reported to be associated with the development of metabolic syndrome (MetS), which is characterized by central obesity, elevated triglycerides (TG), reduced high-density lipoproteins (HDL), impaired fasting blood glucose (FBG), and hypertension. Behçet disease (BD) is a chronic, immuno-inflammatory disease with multisystemic involvement.

Objective

The aim of this study was to investigate the prevalence and risk factors for MetS in patients with BD.

Methods

All patients had BD according to the criteria of the International Study Group. Diagnosis of MetS was established according to National Cholesterol Education Program Adult Treatment Panel III (NCEP ATP III) criteria. Mean waist circumference, body mass index (BMI), FBG, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), total cholesterol, HDL, very low-density lipoprotein (VLDL), low-density lipoprotein (LDL), TG, systolic BP, and diastolic BP were measured and analyzed.

Results

A total of 86 patients and 72 healthy controls were included. MetS was detected in 35.4 % of patients and 20 % of controls (p = 0.04). Patients with BD had a 2.67-fold higher risk for MetS than healthy controls (p < 0.05). Significant risk factors for developing MetS according to multivariate analyses were BD, age, and BMI. Age at onset of the disease, duration of disease, BMI, gastrointestinal system involvement, and neurological involvement were correlated with increased MetS risk (p < 0.05). MetS tended to increase with age and the duration of the disease and was higher in women under the age of 40 years compared with healthy controls in the same age group.

Conclusion

All BD patients should be closely monitored for hypertension, hyperlipidemia, and diabetes mellitus to avoid MetS development.     

Moderation analysis exploring associations between age and mucocutaneous activity in Behçet’s syndrome: A multicenter study from Turkey

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet’s syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement.     

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.     

Dermatological features in Behçet disease-associated vena cava obstruction

Background Behçet disease (BD) is a multisystemic disorder that is classified as vasculitis and can affect all types and sizes of blood vessels. Although vascular diseases are not regarded as essential for a diagnosis of BD, vascular complications can be fatal. Objectives The purpose of this study was to examine the clinical characteristics of 14 patients with BD accompanied by an obstruction of the superior vena cava (VC), the inferior VC, or both, and then determine the dermatological features that indirectly suggest VC obstructions in patients with BD. Methods Among the 3500 patients registered at the BD Specialty Clinic of Severance Hospital from 1997 to 2006, 14 patients were diagnosed with BD‐associated VC obstructions. Their medical records were reviewed. Results Nine of the 14 patients with BD were male, and the age range for disease onset was 11–46 years. Computed tomography revealed superior VC thrombosis in seven patients, inferior VC thrombosis in four patients, and the involvement of both in three patients. The most prominent features in superior VC obstruction were facial oedema (n = 7, 50%) and neck vein distension (n = 6, 43%). Other features included dilated veins in the chest wall, upper body oedema, proptosis, and upper extremity swelling. Patients with an inferior VC obstruction commonly had dilated veins on the abdominal walls. These patients also had abdominal distension and lower extremity swelling. Corticosteroids, colchicine, immunosuppressants, and anticoagulants with or without heparinization were the most common treatment regimens giving good response. Attempts to treat patients with a high dose of steroid pulse therapy were ineffective. Ballooning venoplasty was a useful therapeutic modality for VC thrombosis during the early stage, although this procedure could not be performed in patients with BD with severe thrombosis. Conclusions Several cutaneous features of VC obstruction should be recognized in patients with BD. Early detection might permit an early diagnosis and treatment of occlusion of the VC.     

Successful thalidomide treatment of severe infantile Behçet disease

Abstract: We describe an 11‐month‐old child with giant ulcers of the buccal mucosa, necrosis of the tongue, abdominal tenderness, and severe diarrhea due to Behçet disease. Treatment with thalidomide resulted in prompt recovery of the mucocutaneous lesions and gastrointestinal manifestations.     

Generalized Sweet Syndrome Lesions Associated with Behçet Disease

Sweet syndrome or acute febrile neutrophilic dermatosis is a condition characterized by painful erythematous papules, nodules, and plaques, which demonstrate mature neutrophilic infiltration of the upper dermis histopathologically, and is usually associated with systemic symptoms such as fever and neutrophilia. Generalized Sweet syndrome lesions are usually noted in association with malignancies. Sweet syndrome lesions are rarely seen in patients with Behçet disease and, if present, are usually fewer in number. In this report, we present two female patients who developed generalized Sweet syndrome lesions during follow-up after the diagnosis of Behçet disease. The clinical and histopathologic findings in our patients together with the data in the literature suggest that generalized Sweet syndrome lesions in a patient with clinically inactive Behçet disease should remind the clinician that a strict differential diagnosis needs to be made between Sweet syndrome associated with Behçet disease and the rare cutaneous manifestation of Behçet disease that resembles the lesions of Sweet syndrome.     

Behçet disease in a child--emphasis on cutaneous manifestations

Abstract:  The diagnosis of Behçet disease is based upon clinical criteria because of the lack of pathognomonic laboratory findings. Recurrent episodes of oral and genital ulcerations, skin lesions, and ocular manifestations are seen. The disease may also involve the central nervous system, gastrointestinal tract and, less frequently, the large vessels. In general, manifestations occur in the third or fourth decade of life and are not common in children. Therefore few data concerning this age group have been found in the literature. In this study we report a child with Behçet disease beginning at 1 year of age whose cutaneous manifestations were exuberant acne-like and folliculitis-like lesions, which were crucial for diagnostic confirmation.