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Behçet Disease

Behcets Syndrome

Behcet’s syndrome or Behcet’s disease is a multisystem inflammatory disease characterized by oral aphthous ulcers and a myriad potential systemic manifestations.


Presentation

Behcet’s syndrome is confirmed in the presence of any two of the following criteria:

One of the first symptoms to appear in the case of Behcet’s syndrome is aphthous or herpetiform oral lesions. These lesions have a high recurrence rate and may be found as multiple lesions. Skin lesions are also present in the genital regions. Lesions may be found in scrotum or on the penile shaft. In females lesions develop in the labia, vagina or perineum. These lesions heal with scarring. Tender, erythematosus nodules may develop in the lower extremities and resolve within a few weeks. These nodules are common in females and have a high recurrence rate. In males, acneiform nodules may arise on the trunk and extremities. Ulcerations may occur in axillae, neck, breast and interdigital skin [10].

Oral ulcerations are often followed by ocular manifestations including anterior and posterior uveitis, hypopyon, retinal vasculitis and cystoid macular degeneration. Patients with ocular manifestations may present with blurred vision, photophobia, increased lacrimation and periorbital pain. Ocular symptoms are found to be severe in Iranian and Japanese population. Posterior uveitis may lead to blindness.

As the disease progresses, neurologic manifestations develop. These symptoms often develop 7 to 8 years after the onset of the disease. Most of the neurologic symptoms arise due to the involvement of brain stem. Memory loss is the most common manifestation. Other common neurologic signs include seizures, difficulty in speech, deafness, and clonus.

Pulmonary artery aneurysm may lead to hemoptysis, cough, chest pain and dyspnea [11]. Many small and large blood vessels develop vasculitis and manifestations depend on location of the lesions. Superficial thrombophlebitis with erythema is associated with venous involvement. Deep vein thrombosis is seen in some patients. About 60% of the patients may have arthritis and arthralgia, particularly in knees, ankles, wrists and elbows. Cardiac involvement in the form of coronary vasculitis, pericarditis, myocarditis, endocarditis and diastolic anomalies is also observed in some.

Fever
  • DISCUSSION/CONCLUSION: High fever flares and digestive involvement starting in early childhood seem to be hallmarks of HA20 clinical features.[ncbi.nlm.nih.gov]
  • In December 1998, the patient was admitted with fever, right diplopia, genital ulcers, and aphthous stomatitis. Neurologically diplopia was caused by right abducent nerve paralysis. Brain liquor showed increase in cell count.[doi.org]
  • Clinical Manifestations Oral, eye or genital sores which endure and do not close Pale colored, flat or concave lesions Lassitude Lack of strength Anorexia Abdominal distention and discomfort Loose stools Long, clear urination Cold limbs Maybe low-grade fever[americandragon.com]
Turkish
  • Email: alismjawad1@hotmail.com In 1937, Professor Hulusi Behçet, a Turkish dermatologist, described two cases of relapsing ulceration of the mouth, eye and genitalia, a triple symptom complex that now characterizes the multisystem disorder that was named[web.archive.org]
  • Researchers conducted a genome-wide association study (GWAS) that enrolled 1,209 Turkish people affected by Behcet's disease and 1,278 unaffected Turkish people - all residents of the country.[disabled-world.com]
  • […] aol.com Statistics from Altmetric.com Although much is yet to be discovered about Behçet’s disease, genetic, environmental and infectious factors are thought to play a role in its development Behçet’s disease is a multisystem disorder named after the Turkish[dx.doi.org]
  • The clinical triad of uveitis with recurrent oral and genital ulcers bears the name of Hulusi Behçet, a Turkish dermatologist who described 3 patients who had this triad.[emedicine.com]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • […] chill in the fall Oral or genital sores which are not particularly painful Sores may be accompanied by cold pain The sores are pale red The sores tend to grow hollow Dry, rough, painful eyes A somber, white facial complexion Dizziness Head distention Fatigue[americandragon.com]
  • Side effects of cyclophosphamide include: fatigue (tiredness) nausea (feeling sick) vomiting (being sick) increased vulnerability to infection hair loss – this is usually temporary and your hair should start to grow back once the treatment has been completed[web.archive.org]
  • Some less common problems that can be caused by Behcet disease include: Fever and fatigue Blood clots in the veins of the legs (thrombophlebitis) Obstructed blood vessels causing symptoms in other parts of the body Inflammation of the brain or meninges[saintlukeshealthsystem.org]
  • In the same study, 37 to 47 percent of participants also reported that stress and fatigue made symptoms worse.[medicalnewstoday.com]
Reiter's Syndrome
  • The clinical diagnosis of Reiter's syndrome: ophthalmic and nonophthalmic aspects. Ophthalmology 1986;93 (3) 350- 356 PubMed Google Scholar Crossref 10.[doi.org]
  • Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Crohn's disease and Reiter's syndrome.[healthieryou.com]
  • ’s syndrome Sarcoidosis Seronegative arthropathies Stevens–Johnson syndrome Sweet’s syndrome Syphilis Systemic lupus erythematosus Vogt–Koyanagi–Harada syndrome Behçet’s disease is characterized by episodes of relapses and remissions, and is associated[ncbi.nlm.nih.gov]
Fever of Unknown Origin
  • Other manifestations included Stevens-Johnson-like eruption, fever of unknown origin, and testicular involvement. All of the patients responded to glucocorticoids; two were also treated with colchicine and one was treated with chlorambucil.[pediatrics.aappublications.org]
  • Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early.[ped-rheum.biomedcentral.com]
  • SEP 2000; 12 (5) : 410-414 Majeed-HA Differential diagnosis of fever of unknown origin in children CURRENT-OPINION-IN-RHEUMATOLOGY.[malattierare.regione.veneto.it]
Diarrhea
  • HA20 looks like BD if we consider recurrent oral (87%) and genital (67%) ulcers, arthralgia or arthritis (42%), skin involvement (53%) such as erythema nodosum or abdominal symptoms (60%) such as abdominal pain, digestive ulcers or diarrhea.[ncbi.nlm.nih.gov]
  • Nausea, vomiting, and diarrhea occurred more frequently among the patients treated with apremilast than among those receiving placebo (nausea, 22 patients vs. 10 patients; vomiting, 9 vs. 1; and diarrhea, 12 vs. 2).[doi.org]
  • Symptoms vary from mild abdominal discomfort to severe inflammation of the large intestine and rectum accompanied by diarrhea or bleeding.[web.archive.org]
Abdominal Pain
  • Ovarian vein thrombosis is a rare cause of abdominal pain that should be considered in patients with Behçet disease.[ncbi.nlm.nih.gov]
  • Some symptoms may include mouth and genital ulcers, eye inflammation and reduced vision, skin rashes and lesions, joint swelling, abdominal pain and diarrhea.[sciencedaily.com]
  • Gastrointestinal (GI) tract features include abdominal pain or blood in the stools, which results from lesions similar to those seen in the mouth and genital area.[my.clevelandclinic.org]
Chronic Diarrhea
  • I have gastrointestinal symptoms, ulcers throughout my GI tract, severe abdominal pains and chronic diarrhea, nausea/vomiting, eye inflammation— specifically Uveitis/Iritis, joint inflammation with severe body pain of varying degrees, lung inflammation[globalgenes.org]
  • Wu PS, Chen HL, Yang YH, Jeng YM, Lee PI, Chang MH (2005) Intestinal Behcet disease presenting as neonatal onset chronic diarrhea in an 11-month-old male baby. Eur J Ped 164(8):523–525. doi: 10.1007/s00431-005-1669-0 CrossRef Google Scholar 9.[dx.doi.org]
Vascular Disease
  • ) disease NOS systemic autoimmune disease systemic collagen (vascular) disease Type 1 Excludes autoimmune disease, single organ or single cell-type -code to relevant condition category Systemic connective tissue disorders M35 ICD-10-CM Diagnosis Code[icd10data.com]
  • diseases, Beçhet's disease and other systemic conditions (excluding those secondary to infections) and the use of illicit drugs are reviewed.[web.archive.org]
  • Men tend to have a poorer prognosis. [ 7 ] Mortality is usually low but death may occur as a result of neurological involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.[patient.info]
  • disease so far ( 2 ).[onlinelibrary.wiley.com]
Aphthous Stomatitis
  • A 47-year-old male patient had 10-years history of recurrent aphthous stomatitis and genital ulcer. In December 1998, the patient was admitted with fever, right diplopia, genital ulcers, and aphthous stomatitis.[doi.org]
  • Behçet's syndrome (BS; Adamantiades syndrome) is the association of the triple symptom complex of recurrent aphthous stomatitis (RAS) with genital ulceration, and eye disease (especially iridocyclitis) though a number of other systemic manifestations[ncbi.nlm.nih.gov]
  • Behcet disease aphthous stomatitis genital ulcer iritis Received December 28, 1987. Accepted February 25, 1988.[pediatrics.aappublications.org]
  • Symptoms The most frequent sign is aphthous stomatitis, (inflammation of the mucosa of the mouth) with the lesions healing in a few days to a month, but recurring. Similar genital lesions recur less frequently.[healthcentral.com]
Recurrent Oral Ulceration
  • Behçet disease is a multisystem vasculitis characterised by recurrent oral ulceration in conjunction with other manifestations.[ncbi.nlm.nih.gov]
  • Based on these criteria, a diagnosis of Behçet disease requires recurrent oral ulceration and at least 2 additional criteria, including recurrent genital ulcers, ocular lesions, skin lesions, and a positive pathergy test.[emedicine.com]
Sore Mouth
  • Mouth soresMouth sores (known as oral aphthosis and aphthous stomatitis) affect almost all patients with Beh et's disease. They are often the first symptom that a person notices and may occur long before any other symptoms appear.[healthieryou.com]
Arthritis
  • Pain intensity ( p 0.000), NHP ( p 0.004), and HAQ ( p 0.003) scores were significantly higher in BD patients with arthritis than those without arthritis.[oadoi.org]
  • Abstract The spondyloarthropathies comprise ankylosing spondylitis (AS), reactive arthritis, psoriatic arthritis (PsA) and arthritis associated with inflammatory bowel disease.[ncbi.nlm.nih.gov]
Arthralgia
  • Features such as arthralgia and leucocytoclastic vasculitis suggest an immune-complex mediated basis, which is supported by finding circulating immune complexes and, although the antigen responsible is unidentified, heat shock proteins have been implicated[ncbi.nlm.nih.gov]
  • Symptoms · Skin irritation · Hereditary · Arthritis/Arthralgia · Bowels – GI manifestations include abdominal pain, nausea, and diarrhea with or without blood, and they often involve the ileocecal valve.[powerofpain.org]
Joint Swelling
  • swelling and pain … has struggled with what was diagnosed as Behcet's disease, a form of vasculitis, which causes lesions, mouth blisters and joint pain. — Brad Townsend, The Dallas Morning News, 14 May 2012[merriam-webster.com]
  • Some symptoms may include mouth and genital ulcers, eye inflammation and reduced vision, skin rashes and lesions, joint swelling, abdominal pain and diarrhea.[sciencedaily.com]
  • Genital sores Eye disease: Redness Blurry vision Light sensitivity Watery eyes Joint swelling and pain—usually does not cause permanent joint damage Skin problems such as sores that: May appear as red, raised sores or bumps, or may be flat Usually appear[cancercarewny.com]
  • Joint swelling and pain often affect the knees in people with Behcet's disease. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own. Vascular system.[mayoclinic.org]
  • swelling Brain and spinal cord: Fever Headache Stiff neck Confusion Personality changes Memory loss Trouble speaking Digestive system : Stomach pain Diarrhea Bleeding from sores in the stomach and intestines Sometimes Behcet's also affects other organs[webmd.com]
Blurred Vision
  • It can present with blurred vision, floaters, eye pain, redness and sensitivity to light. In addition to Behcet's Disease, there are many infectious and non-infectious causes which may result in posterior uveitis.[behcets.com]
  • Common symptoms include: genital and mouth ulcers red, painful eyes and blurred vision acne -like spots headaches painful, stiff and swollen joints In severe causes, there is also a risk of serious and potentially life-threatening problems, such as permanent[web.archive.org]
  • Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet’s disease and can cause blurred vision, pain, and redness.[pvfla.org]
  • Symptoms of eye inflammation include pain, blurred vision, tearing, redness, and pain when looking at bright lights.[medicinenet.com]
Eye Pain
  • The most common symptoms are Sores in the mouth Sores on the sex organs Other skin sores Swelling of parts of the eye Pain, swelling and stiffness of the joints More serious problems can include meningitis, blood clots, inflammation of the digestive system[nlm.nih.gov]
  • The most common symptoms are sores in the mouth sores on the sex organs other skin sores swelling of parts of the eye pain, swelling and stiffness of the joints more serious problems can include meningitis, blood clots, inflammation of the digestive system[icd9data.com]
Photophobia
  • The retina may become inflamed resulting in blurred vision, abnormal sensitivity to light (photophobia), and/or, inflammation of the thin membranous layer of blood vessels behind the retina (chorioretinitis).[web.archive.org]
  • Patients with ocular manifestations may present with blurred vision, photophobia, increased lacrimation and periorbital pain. Ocular symptoms are found to be severe in Iranian and Japanese population. Posterior uveitis may lead to blindness.[symptoma.com]
  • Uveitis can result in blurry vision and an extreme sensitivity to light (photophobia). Rarely, inflammation can also cause eye pain and redness. If untreated, the eye problems associated with Behçet disease can lead to blindness.[ghr.nlm.nih.gov]
  • Other ocular manifestations include hypopyon, retinal vasculitis, retinal haemorrhage, blurred vision and photophobia. Neurological: central nervous system (CNS) involvement occurs late in the disease.[patient.info]
  • Clinical Manifestations During the mid to later stages of the disease or due to prolonged steroid use, there oral and genital sores that do not heal The sores are dark red and insidiously painful Dry, rough eyes Dark orbits of the eyes Blurred vision Photophobia[americandragon.com]
Retinal Hemorrhage
  • Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology.[ncbi.nlm.nih.gov]
  • The most frequent fundus changes were vascular sheathing in 61 eyes (23.7%), optic atrophy in 46 (17.9%), macular edema in 29 (11.3%), retinal hemorrhage in 23 (9%), macular scar in 21 (8.2%), optic disc paleness in 19 (7.4%), retinal edema in 17 (6.6%[doi.org]
  • Other fundoscopic findings include vascular sheathing (23.7%), retinal hemorrhage (9%), macular edema (11.3%), branch retinal vein occlusion (5.8%), and retinal edema (6.6%).[en.wikipedia.org]
Red Eye
  • She specifically denies genital ulcers or genital scars, fever, chills, night sweats, alopecia, photosensitivity, Raynaud's phenomena, red eyes, nasal ulcers, cough, dyspnea, hemoptysis, pleuritic chest pain, difficulty swallowing, nausea, vomiting, dysuria[webeye.ophth.uiowa.edu]
Ulcer
  • Behçet disease (BD) is a recurrent vasculitis characterized by oral and genital mucous membrane ulcers, uveitis, and skin lesions but only rarely leg ulcers.[ncbi.nlm.nih.gov]
  • ulcers at baseline; all were free from genital ulcers at week 12.[doi.org]
Skin Lesion
  • Behçet disease is a multisystemic chronic inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions.[ncbi.nlm.nih.gov]
Papule
  • […] aneurysms or severe neurological complications Case reports 34 year old man with aphthous stomatitis, then painful erythematous nodules on lower extremities ( Dermatol Online J 2010;16:18 ) Clinical images Images hosted on other servers: Erythematous papules[pathologyoutlines.com]
  • Pathergy test: minor skin trauma induces an inflammatory papule or pustule after 24-48 hours. This is positive in up to 60% of patients. Imaging studies, including CT and/or MRI scan, may be undertaken in some patients.[patient.info]
  • […] the following 4 "hallmark" symptoms: eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous) genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men) pathergy reaction (papule[en.wikipedia.org]
  • A specific cutaneous irritability is frequent with papules on site of injections.[dx.doi.org]
Acne Vulgaris
  • vulgaris and rheumatoid arthritis.[patient.info]
  • Mild forms of papulopustular or acne-like lesions are treated first by topical measures as used in acne vulgaris.[ard.bmj.com]
  • In contrast to acne vulgaris, pseudofolliculitis and acneiform nodules can appear all over the body and they are not always hair follicle-associated.[dx.doi.org]
  • A folliculitis-like rash, resembling acne vulgaris, appears not only on the face, but also on the neck, chest, back, and hairline of patients [ 6 ].[ncbi.nlm.nih.gov]
  • Thus, topical measures as used in acne vulgaris are sufficient. Colchicine should be preferred when the dominant lesion is erythema nodosum. Leg ulcers in BD might have different causes. Treatment should be planned accordingly.[doi.org]
Skin Ulcer
  • […] inflammation with venous and arterial vasculitis (possibly obliterative) Positive pathergy test Aphthous ulcers Genital ulcers Erythema nodosum Dermatographia Symptoms of Behcet's Photophobia Vision loss Oral ulcers Genital ulcers Arthritis Headache Skin[webeye.ophth.uiowa.edu]
Headache
  • Anticoagulant and steroid therapy were effective to relieve the headache, and to improve the constricted visual field temporarily. By MRA, blood flow was confirmed in the sagittal sinus.[ncbi.nlm.nih.gov]
  • Headaches In cases of Behçet’s disease, headaches are usually treated in the same way as migraines.[web.archive.org]
  • Headaches In cases of Behçet's disease, headaches are usually treated in the same way as migraines.[nhs.uk]
Confusion
  • Recurrent attacks of confusion or mental dullness were found in two patients, and high cortical dysfunction persisted on follow-up for some time.[ncbi.nlm.nih.gov]
  • This can result in confusion and coma. Typically these features occur later in the disease course, years after the diagnosis.[medicinenet.com]
  • . · Nervous System Symptoms · Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss · Neurological system – CNS involvement[powerofpain.org]
  • The neurologic features are variable and include headache, confusion, strokes, personality changes and memory loss. Central nervous system symptoms in Behcet's Disease may be due to aseptic meningitis or lesions resulting in focal brain dysfunction.[behcets.com]
  • Genitals: Sores on the scrotum in men Sores on the vulva in women Skin : Bumps that look like acne Red lumps on the legs and ankles Joints: Pain in the knees, ankles, wrists, and elbows Joint swelling Brain and spinal cord: Fever Headache Stiff neck Confusion[webmd.com]
Penile Lesion
  • OCT 2000; 21 (10) : 905-907 Seftel-AD; Sheahan-MG; Scolieri-M Desquamative penile lesion as a manifestation of Behcets disease JOURNAL-OF-UROLOGY.[malattierare.regione.veneto.it]
  • They may also affect the epididymis; penile lesions are less frequent [ 1 ]. In females, vulvar, vaginal and cervical lesions are especially common [ 5 ]. Rarely, deep vaginal lesions may perforate the bladder resulting in fistulae [ 16, 17 ].[doi.org]
Dysuria
  • She specifically denies genital ulcers or genital scars, fever, chills, night sweats, alopecia, photosensitivity, Raynaud's phenomena, red eyes, nasal ulcers, cough, dyspnea, hemoptysis, pleuritic chest pain, difficulty swallowing, nausea, vomiting, dysuria[webeye.ophth.uiowa.edu]

Workup

Imaging techniques like MRI, CT scan, and radiography are useful in identifying tissue swelling, effusions and areas of ischemia. Brain MRI is necessary when there is an involvement of CNS. MRI helps in locating focal lesions of cerebral vasculopathy. Aneurysms can be detected using angiograms. Echocardiography is an imaging method useful in diagnosing ventricular thrombi.

There are no specific laboratory tests for the diagnosis of Behcet’s syndrome. Patients often have normal levels of serum components. Some of the complements may show a change when ocular or skin manifestations arise. Few patients may show increased ESR and C-reactive protein levels. Anticardiolipin antibodies are reported in some patients.

Gastrointestinal lesions are diagnosed using endoscopy. Eye examination is essential when ocular manifestations are obvious. Retinal vessels are evaluated using fluorescein angiography. Positive pathergy test is indicative of Behcet’s disese as skin prick may trigger nodule or pustule formation within 2 days. Perivascular infiltration and vasculitis can be identified using sample biopsy.

Elevated Sedimentation Rate
  • Patients received medical evaluation of a series of laboratory blood tests (peripheral blood count, calcium, sodium, potassium, chloride, elevated sedimentation rate, C-reactive protein, glucose, serum angiotensin-converting enzyme, rheumatoid factor,[ncbi.nlm.nih.gov]
Colitis
  • Behçet disease with gastrointestinal involvement in ulcerative colitis (UC) patient has been reported in just 1 previous case report, but, which can not be diagnosed as definite intestinal BD based on Korean novel diagnositic criteria due to lacking the[ncbi.nlm.nih.gov]
  • Symptoms vary from mild gastrointestinal discomfort to ulcerative colitis or regional enteritis and malabsorption problems. Arthritis occurs in about two-thirds of patients, most commonly affecting the knees and ankles.[healthcentral.com]
  • Kim JS, Lim SH, Choi IJ, Moon H, Jung HC, Song IS et al (2000) Prediction of the clinical course of Behcet’s colitis according to macroscopic classification by colonoscopy.[dx.doi.org]
Colonic Ulcer
  • A 61-year-old man developed sudden hematochezia due to bleeding from multiple small colonic ulcers; AL-amyloid deposition was found on immunohistochemical examination of biopsy specimen of colonic ulcer.[ncbi.nlm.nih.gov]
  • Temporal changes in the clinical type or diagnosis of Behçets’s colitis in patients with aphthoid or punched-out colonic ulcerations. J Korean Med Sci. 1991; 6 313-318 23 McHugh J B, Appelman H D, McKenna B J.[dx.doi.org]
HLA-B5
  • BS mainly affects young adult males, and there is an association with HLA-B5 and HLA-B51 (B5101).[ncbi.nlm.nih.gov]
  • A statistically significant increased incidence of HLA-B5 or Bw51 was noted in the patients as compared with the normal control subjects. No significant difference was observed in the distribution of other HLA antigens.[doi.org]
  • BS mainly affects young adult males, and there is an association with HLAB5 and HLA‐B51 (B5101).[dx.doi.org]
HLA-Bw51
  • On the basis of HLA studies, it seems likely that the susceptibility genes to Behçet's disease closely linked to HLA-Bw51 may have been spread by the old nomadic tribes or the Turks via the Silk Route.[doi.org]
  • Google Scholar Ohno S, Ohguchi M, Hirose S, Matsuda H, Wakisaka A, Aizawa M: Close association of HLA-Bw51 with Behcet's disease. Arch Ophthalmol. 1982, 100 (9): 1455-1458. 10.1001/archopht.1982.01030040433013.[dx.doi.org]

Treatment

Treatment modality depends on the organ involved and severity of the symptoms. Topical steroids and sucralfate solution are recommended in mild cases of oral and genital ulcerations. Colchicine is used to prevent relapse of mucocutaneous lesions [12]. Severe lesions are treated with systemic corticosteroids, azathioprine, dapsone and interferon-alpha. Mild ocular manifestations are treated with azathioprine. In severe cases of ocular symptoms, cyclosporine A or infliximab is used in combination with corticosteroids. Interferon-alpha is also recommended in severe cases of ocular manifestations. Systemic corticosteroids and azathioprine are suggested for gastrointestinal ulcerations. For cutaneous manifestations, colchicine and dapsone are used. Thrombi in vessels are treated with systemic anticoagulants with corticosteroids or azathioprine. Aneurysms in pulmonary vessels need cyclophosphamide with corticosteroids.

Those presenting with intestinal stenosis, severe bleeding, perforations and fistula, may need surgery. Resection is suggested for ischemic damage with vasculitis. Surgery is also recommended in coronary thrombosis, endocardial fibrosis or ventricular aneurysms. Some ocular manifestations like glaucoma, cataract and retinal detachment may require surgical intervention. Clots and aneurysms in the CNS are also improved with surgery.

Prognosis

In general, prognosis depend on the clinical manifestations of the disease. Treatment, rest and exercise are the best steps to improve the symptoms of the condition. Effective treatment helps to prevent exacerbations. Patients may enter a period of remission in most of the cases, while serious complications may arise in some. Those who develop retinal vasculitis, vascular aneurysms, and neurological manifestations may not have a good prognosis. Retinal vasculitis may lead to blindness while aneurysms in the blood vessels may rupture.

Etiology

Etiology of the disease is not defined. Viral etiology, though accepted earlier, was disproved over the years. New theories propose various other factors as the causative feature including bacterial infection, exposure to chemicals and, most recent, autoimmune reaction. Clinical manifestations are caused by immune responses that lead to vasculitis of small and medium sized blood vessels. The reaction is also thought to initiate inflammation of epithelium mediated by hyperactive T lymphocytes and plasma cells. Increased function of neutrophils noted in patients is also implicated in the development of clinical manifestations.

A genetic component, particularly HLA-B51, has an association with this syndrome. Interleukin 10 (IL-10) and IL-23 are also reported to have strong influences in the development of Behcet’s disease [2]. Patients with active form of Becet’s syndrome have elevated levels of IL-6, while IL-10 remains lower than normal [3].

Epidemiology

This syndrome exists worldwide, but with distinct differences in the regions. Worldwide prevalence studies show the estimate to be 1:10,000. Behcet’s disease is a more prevalent condition in the Far East, Middle East and Mediterranean countries like Iran, Turkey and Israel. In Turkey the estimate is in the range of 80-370 in a population of 100,000. Western countries show a prevalence ranging from 5 to 6.4 per 100,000 population [4]. Incidence was found to be higher among males in the Mediterranean region, and this is particularly true for men in the age group of 30-40 years. In Eastern countries the male to female ratio was found to be 1:1, while in Japan and Korea females are more commonly affected. Although there is a variation in the sex ratios from different regions, the course of the disease is more severe among males. Average onset age (25-35 years) was similar in men and women, some cases have been reported in childhood as well. It is rare for the disease to occur before school age. In Turkey the mean age for the onset of Behcet’s disease is 11.7 years.

Sex distribution
Age distribution

Pathophysiology

A number of theories have been put forward to explain pathogenesis of this disease. The latest and most accepted one is the autoimmune pathogenesis of Behcet’s syndrome. Infectious agents like Streptococcus, Staphylococcus species, and herpes simplex virus (HSV) may trigger an immune reaction. Heat shock proteins (HSP) are also suspected as triggers of cross-reactive immune reaction that lead to Behcet’s disease. In high-risk populations, including Turkey and Japan, HSPs bring out an enhanced activation of lymphocytes resulting in a proliferative response, characteristic of the disease [5]. Different ethnic populations show increased T- and B-cell responses to these proteins and hence HSPs may be pathophysiologically related to the development of Behcet’s syndrome.

T-cells and neutrophils undergo inflammatory tissue infiltration resulting in vasculitic and vasculopathic lesions that are common manifestations in the disease [6]. The specific roles of the neutrophils in the disease is not fully characterized. Chemotaxis, active oxygen production, phagocytosis, and infiltration are all implicated in the course of this syndrome [7]. Many recent evidences suggest a critical role for nitric oxide, a free oxygen radical released by endothelial cells upon stimulation by cytokines, in the development and progression of Behcet’s disease. Increased production of nitrogen oxide is associated with increased susceptibility to the syndrome [8].

Behcet’s disease may be seen in a family, which suggests genetic pathophysiology of the disease. Presence of HLA-B51/HLA-B5 genetic structure is associated with increased risk of this syndrome [9]. But these genes are not shown to have any effect on the severity of the disease.

Prevention

No known preventive measures exist for Behcet’s syndrome.

Summary

Behcet’s syndrome or Behcet’s disease is a multisystemic inflammatory disease first described by Turkish dermatologist Hulusi Behçet in 1937. It was initially described as a ‘triple symptoms complex’ with characteristic symptoms – aphthous ulceration, genital ulceration and inflammatory eye disease [1]. The condition may cause heterogeneous clinical manifestations including ocular, mucocutaneous, neurologic, cardiovascular, pulmonary, renal and gastrointestinal symptoms. Symptoms often cluster together, but the manifestations may vary from region to region.

The precise etiology of the disease is not yet known. Highest incidence of Behcet’s syndrome is reported from the Middle East, Mediterranean and the Far East. It is a rare syndrome and young adult men are more commonly affected by the condition. Family aggregation of the syndrome, affecting three brothers in a family, is also reported. A number of etiologic factors are thought to be associated with the development of Behcet’s syndrome, the latest and most accepted one being autoimmune reaction. Treatment is based on clinical manifestations of the condition.

Patient Information

Behcet’s syndrome or Behcet’s disease is an autoimmune disease characterized by triad of symptoms – oral ulcers, sores in the genital area and inflammation in parts of the eye. Many tissues and blood vessels may become inflamed, resulting in the different symptoms of the disease. Symptoms may vary from person to person depending on the location of inflammation and the organ involved. Some of the areas that are commonly affected by the disease include mouth, skin, genitals, eyes, blood vessels, digestive system, brain, and joints.

The actual cause of the disease is not fully characterized. A combination of environmental and genetic factors are thought to play an important role in the development of this condition. This syndrome is more common among men and women in the age group of 30 to 40 years. People from Far East, Mediterranean, and Middle East have an increased risk of developing this syndrome. Certain genes are also known to be associated with the disease. Behcet’s syndrome is seen in both men and women, but is more severe in men.
Confirmatory diagnosis depends on the presence of any of the two symptoms given below:

  • Mouth sores
  • Recurrent genital sores
  • Eye problems 
  • Skin sores
  • Positive pathergy test – In this test, a sterile needle is inserted into the skin and if small pustules are formed within 2 days, it is considered to be a positive test for increased immune activity. 

Complete cure for the disease is not available. Treatment often depends on the severity of the symptoms and the organ involved. Topical creams are used for sores in the skin and genital area. Eye drops containing corticosteroids are useful in improving the symptoms of the eye. When topical medications are not effective, oral medications are recommended. Medications are used to suppress the immune system and also to control inflammation. In some cases medications are suggested to regulate the response of the immune system. Prompt treatment, adequate rest, good diet and exercise go a long way in controlling the symptoms of Behcet’s syndrome.

References

Article

  1. Behcet H. Some observations on the clinical picture of the so-called triple syndrome complex. Dermatologia. 1940;81:73-83.
  2. Ozen S, Eroglu FK. Pediatric-onset Behçet disease. Curr Opin Rheumatol. 2013;25(5):636-42.
  3. Talaat RM, Ashour ME, Bassyouni IH, Raouf AA. Polymorphisms of interleukin 6 and interleukin 10 in Egyptian people with Behcet's disease. Immunobiology. 2014; 219(8):573-582.
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Last updated: 2019-07-11 22:01