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Behcet Disease

Updated : August 17, 2023





Background

Behcet’s disease, also known as Behcet syndrome, is a rare autoimmune disorder that affects multiple organ systems, including the skin, eyes, mouth, and genitalia. The disease is named after Hulusi Behcet, a Turkish dermatologist who first described the condition in 1937. The exact cause of Behcet’s disease is unknown, but it is thought to be related to an abnormal immune response that leads to inflammation of blood vessels, resulting in damage to various organs.

Behcet’s disease is more common in the Middle East, Asia, and the Mediterranean regions, but it can occur in any population. The disease usually affects people in their 20s and 30s, and it affects both men and women equally. The symptoms of Behcet’s disease can be variable, and the disease can be difficult to diagnose, leading to delays in treatment. There is no cure for Behcet’s disease, but the symptoms can be managed with medications and other supportive measures.

Epidemiology

Behcet’s disease is a rare disorder, and its prevalence varies widely among different populations. The disease is more common in the Middle East, Asia, and the Mediterranean regions, where the reported prevalence ranges from 20 to 370 cases per 100,000 individuals. The incidence of Behcet’s disease is highest in Turkey, Iran, and Japan.

Behcet’s disease is relatively uncommon in North America, Europe, and Africa, with reported prevalence rates ranging from 0.5 to 7.5 cases per 100,000 individuals. The disease affects both men and women equally, and it typically presents in the third or fourth decade of life. However, the disease can occur at any age, including during childhood.

Behcet’s disease is more common in people of certain ethnic backgrounds, including those of Turkish, Iranian, and Japanese descent. The exact reasons for these ethnic differences in the prevalence of Behcet’s disease are unclear, but genetic and environmental factors are thought to play a role. Overall, Behcet’s disease is a rare disorder, but its prevalence varies widely among different populations. The disease is more common in certain regions and ethnic groups and typically affects people in their 20s and 30s.

Anatomy

Pathophysiology

The pathophysiology of Behcet’s disease is not fully understood, but it is believed to be related to an abnormal immune response that leads to inflammation and damage to blood vessels in various organs. It is hypothesized that the triggering event for Behcet’s disease is an abnormal immune response to an infectious or environmental trigger, which leads to the activation of immune cells and the production of pro-inflammatory cytokines.

These cytokines cause endothelial damage and activation, leading to the recruitment of leukocytes to the site of inflammation, resulting in the formation of immune complexes and vascular inflammation. Behcet’s disease is associated with the presence of HLA-B51, an allele of the major histocompatibility complex (MHC) that plays a role in presenting antigens to immune cells.

It is thought that the presence of this allele may predispose individuals to an abnormal immune response to environmental triggers, leading to the development of Behcet’s disease. The vascular inflammation seen in Behcet’s disease can lead to a variety of clinical manifestations, including oral and genital ulcers, skin lesions, and ocular inflammation.

The inflammation can also affect the joints, gastrointestinal tract, and central nervous system, leading to a range of other symptoms. The pathophysiology of Behcet’s disease is complex and involves multiple genetic and environmental factors. Further research is needed to fully understand the mechanisms underlying the disease and to develop more effective treatments.

Etiology

The exact cause of Behcet’s disease is unknown, but it is thought to be related to a combination of genetic and environmental factors. Genetic factors are thought to play a role in the development of Behcet’s disease. Certain genetic variations, including the HLA-B51 allele of the major histocompatibility complex, have been associated with an increased risk of developing the disease. Other genetic variations involved in the immune system response and inflammation have also been identified as potential risk factors for Behcet’s disease.

Environmental factors may also contribute to the development of Behcet’s disease. Infections, such as Streptococcus sanguis and herpes simplex virus, have been proposed as potential triggers of the abnormal immune response that leads to Behcet’s disease. Other environmental factors, such as exposure to toxins or pollutants, may also contribute to the development of the disease. Some researchers believe that Behcet’s disease is an autoimmune disorder in which the body’s immune system mistakenly attacks healthy tissues and organs, leading to inflammation and damage.

However, this theory has yet to be definitively proven, and the exact mechanisms underlying the disease still need to be fully understood. Overall, the etiology of Behcet’s disease is complex and likely involves a combination of genetic and environmental factors. Further research is needed to fully understand the underlying causes of the disease and to develop more effective treatments.

Genetics

Prognostic Factors

The prognosis of Behcet’s disease can vary widely depending on the severity and location of the symptoms and the response to treatment. In general, the disease tends to follow a chronic relapsing and remitting course, with periods of active disease followed by periods of remission. While Behcet’s disease is a chronic condition, it is not typically life-threatening.

However, the disease can cause significant morbidity and disability if left untreated or if not managed appropriately. Complications of Behcet’s disease can include vision loss, joint damage, gastrointestinal bleeding, and neurological deficits. The prognosis is generally better for patients with milder forms of the disease, such as those with primarily oral or genital ulcers, compared to those with more severe forms of the disease, such as those with significant eye involvement or neurological symptoms.

Early diagnosis and treatment are important for improving the prognosis and preventing complications associated with the disease. Regular monitoring and follow-up with a healthcare provider, including ophthalmologic monitoring in patients with eye involvement, is important for ensuring that the disease is appropriately managed, and complications are detected and treated early.

Clinical History

Clinical history

Behcet’s disease is a systemic inflammatory disorder that can affect multiple organs and systems. The clinical history of Behcet’s disease may vary widely depending on the specific manifestations of the disease in each patient. However, some common features of the disease may include:

Oral and genital ulcers: Recurrent painful ulcers in the mouth and genital areas are a hallmark feature of Behcet’s disease. These ulcers may be round or oval and can be deep and persistent.

Skin lesions: Behcet’s disease may cause a variety of skin lesions, including erythema nodosum, papulopustular lesions, and nodules. These lesions may be painful and may appear on the trunk, arms, legs, and face.

Eye inflammation: Behcet’s disease may cause inflammation in the eyes, which can lead to pain, redness, and sensitivity to light. In severe cases, it can lead to vision loss.

Joint pain and inflammation: Behcet’s disease can cause joint pain, swelling, and stiffness. The joints most commonly affected are the knees, ankles, and wrists.

Gastrointestinal symptoms: Behcet’s disease can cause abdominal pain, diarrhea, and bleeding. It can also lead to ulcerations and perforations in the digestive tract.

Neurological symptoms: Behcet’s disease can cause a range of neurological symptoms, including headache, dizziness, and seizures.

The symptoms of Behcet’s disease can come and go and may vary in severity over time. The disease may also progress slowly over time, leading to a worsening of symptoms and potential complications. A thorough medical history and physical examination, as well as appropriate laboratory testing, are necessary to make an accurate diagnosis of Behcet’s disease.

Physical Examination

Physical examination

A physical examination is an important part of the diagnostic process for Behcet’s disease. The examination may reveal various signs and symptoms, depending on the specific organs and systems affected by the disease. Some of the physical findings that may be seen in Behcet’s disease include:

  • Oral ulcers: round or oval ulcers on the tongue, inside of the cheeks, or on the lips.
  • Genital ulcers: painful ulcers on the penis, vulva, or vagina.
  • Skin lesions: red or purple, raised or flat lesions on the skin, including erythema nodosum, papulopustular lesions, and nodules.
  • Eye inflammation: redness, pain, and sensitivity to light, along with inflammation in the eye that can be seen on an eye exam.
  • Joint inflammation: swelling, redness, and tenderness in the joints, most commonly in the knees, ankles, and wrists.
  • Gastrointestinal symptoms: abdominal tenderness, bloating, and diarrhea, as well as signs of inflammation on imaging tests.
  • Neurological symptoms: signs of neurological involvement, such as headache, dizziness, and altered mental status.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

The clinical manifestations of Behcet’s disease can vary widely, and as a result, the differential diagnosis may include a range of other conditions that share similar symptoms. Some of the conditions that may be considered in the differential diagnosis of Behcet’s disease include:

  • Oral and genital ulcers: recurrent aphthous stomatitis, herpes simplex virus infection, syphilis, Crohn’s disease, and other inflammatory bowel diseases
  • Skin lesions: erythema nodosum, erythema multiforme, cutaneous vasculitis, lupus erythematosus, and pyoderma gangrenosum
  • Eye inflammation: uveitis, scleritis, acute angle-closure glaucoma, and herpes simplex virus infection
  • Joint pain and inflammation: rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and systemic lupus erythematosus
  • Neurological symptoms: multiple sclerosis, meningitis, and stroke.

It is important to note that Behcet’s disease is a diagnosis of exclusion, meaning that other potential causes of the symptoms must be ruled out before a definitive diagnosis can be made. A thorough medical history, physical examination, and laboratory testing may be necessary to make an accurate diagnosis and to differentiate Behcet’s disease from other conditions with similar symptoms.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Treatment of Behcet’s disease aims to control the symptoms and prevent complications associated with the disease. The treatment plan may vary depending on the severity and location of the symptoms and may include medications and lifestyle modifications. Some treatment options for Behcet’s disease include:

Nonsteroidal anti-inflammatory drugs (NSAIDs): These medications can be effective in treating joint pain and inflammation associated with Behcet’s disease.

Corticosteroids: Steroid medications can be effective in reducing inflammation associated with Behcet’s disease, especially in more severe cases.

Immunomodulatory drugs: Medications that suppress the immune system, such as azathioprine, methotrexate, and cyclosporine, can be effective in treating severe cases of Behcet’s disease.

Biologic agents: These medications target specific parts of the immune system and can be effective in treating severe or refractory Behcet’s disease.

Colchicine: This medication can be effective in reducing the frequency and severity of oral and genital ulcers associated with Behcet’s disease.

Lifestyle modifications: Avoiding triggers that may exacerbate symptoms, such as stress, certain foods, and smoking, can help manage symptoms of Behcet’s disease.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK470257/

Behcet Disease

Updated : August 17, 2023




Behcet’s disease, also known as Behcet syndrome, is a rare autoimmune disorder that affects multiple organ systems, including the skin, eyes, mouth, and genitalia. The disease is named after Hulusi Behcet, a Turkish dermatologist who first described the condition in 1937. The exact cause of Behcet’s disease is unknown, but it is thought to be related to an abnormal immune response that leads to inflammation of blood vessels, resulting in damage to various organs.

Behcet’s disease is more common in the Middle East, Asia, and the Mediterranean regions, but it can occur in any population. The disease usually affects people in their 20s and 30s, and it affects both men and women equally. The symptoms of Behcet’s disease can be variable, and the disease can be difficult to diagnose, leading to delays in treatment. There is no cure for Behcet’s disease, but the symptoms can be managed with medications and other supportive measures.

Behcet’s disease is a rare disorder, and its prevalence varies widely among different populations. The disease is more common in the Middle East, Asia, and the Mediterranean regions, where the reported prevalence ranges from 20 to 370 cases per 100,000 individuals. The incidence of Behcet’s disease is highest in Turkey, Iran, and Japan.

Behcet’s disease is relatively uncommon in North America, Europe, and Africa, with reported prevalence rates ranging from 0.5 to 7.5 cases per 100,000 individuals. The disease affects both men and women equally, and it typically presents in the third or fourth decade of life. However, the disease can occur at any age, including during childhood.

Behcet’s disease is more common in people of certain ethnic backgrounds, including those of Turkish, Iranian, and Japanese descent. The exact reasons for these ethnic differences in the prevalence of Behcet’s disease are unclear, but genetic and environmental factors are thought to play a role. Overall, Behcet’s disease is a rare disorder, but its prevalence varies widely among different populations. The disease is more common in certain regions and ethnic groups and typically affects people in their 20s and 30s.

The pathophysiology of Behcet’s disease is not fully understood, but it is believed to be related to an abnormal immune response that leads to inflammation and damage to blood vessels in various organs. It is hypothesized that the triggering event for Behcet’s disease is an abnormal immune response to an infectious or environmental trigger, which leads to the activation of immune cells and the production of pro-inflammatory cytokines.

These cytokines cause endothelial damage and activation, leading to the recruitment of leukocytes to the site of inflammation, resulting in the formation of immune complexes and vascular inflammation. Behcet’s disease is associated with the presence of HLA-B51, an allele of the major histocompatibility complex (MHC) that plays a role in presenting antigens to immune cells.

It is thought that the presence of this allele may predispose individuals to an abnormal immune response to environmental triggers, leading to the development of Behcet’s disease. The vascular inflammation seen in Behcet’s disease can lead to a variety of clinical manifestations, including oral and genital ulcers, skin lesions, and ocular inflammation.

The inflammation can also affect the joints, gastrointestinal tract, and central nervous system, leading to a range of other symptoms. The pathophysiology of Behcet’s disease is complex and involves multiple genetic and environmental factors. Further research is needed to fully understand the mechanisms underlying the disease and to develop more effective treatments.

The exact cause of Behcet’s disease is unknown, but it is thought to be related to a combination of genetic and environmental factors. Genetic factors are thought to play a role in the development of Behcet’s disease. Certain genetic variations, including the HLA-B51 allele of the major histocompatibility complex, have been associated with an increased risk of developing the disease. Other genetic variations involved in the immune system response and inflammation have also been identified as potential risk factors for Behcet’s disease.

Environmental factors may also contribute to the development of Behcet’s disease. Infections, such as Streptococcus sanguis and herpes simplex virus, have been proposed as potential triggers of the abnormal immune response that leads to Behcet’s disease. Other environmental factors, such as exposure to toxins or pollutants, may also contribute to the development of the disease. Some researchers believe that Behcet’s disease is an autoimmune disorder in which the body’s immune system mistakenly attacks healthy tissues and organs, leading to inflammation and damage.

However, this theory has yet to be definitively proven, and the exact mechanisms underlying the disease still need to be fully understood. Overall, the etiology of Behcet’s disease is complex and likely involves a combination of genetic and environmental factors. Further research is needed to fully understand the underlying causes of the disease and to develop more effective treatments.

The prognosis of Behcet’s disease can vary widely depending on the severity and location of the symptoms and the response to treatment. In general, the disease tends to follow a chronic relapsing and remitting course, with periods of active disease followed by periods of remission. While Behcet’s disease is a chronic condition, it is not typically life-threatening.

However, the disease can cause significant morbidity and disability if left untreated or if not managed appropriately. Complications of Behcet’s disease can include vision loss, joint damage, gastrointestinal bleeding, and neurological deficits. The prognosis is generally better for patients with milder forms of the disease, such as those with primarily oral or genital ulcers, compared to those with more severe forms of the disease, such as those with significant eye involvement or neurological symptoms.

Early diagnosis and treatment are important for improving the prognosis and preventing complications associated with the disease. Regular monitoring and follow-up with a healthcare provider, including ophthalmologic monitoring in patients with eye involvement, is important for ensuring that the disease is appropriately managed, and complications are detected and treated early.

Clinical history

Behcet’s disease is a systemic inflammatory disorder that can affect multiple organs and systems. The clinical history of Behcet’s disease may vary widely depending on the specific manifestations of the disease in each patient. However, some common features of the disease may include:

Oral and genital ulcers: Recurrent painful ulcers in the mouth and genital areas are a hallmark feature of Behcet’s disease. These ulcers may be round or oval and can be deep and persistent.

Skin lesions: Behcet’s disease may cause a variety of skin lesions, including erythema nodosum, papulopustular lesions, and nodules. These lesions may be painful and may appear on the trunk, arms, legs, and face.

Eye inflammation: Behcet’s disease may cause inflammation in the eyes, which can lead to pain, redness, and sensitivity to light. In severe cases, it can lead to vision loss.

Joint pain and inflammation: Behcet’s disease can cause joint pain, swelling, and stiffness. The joints most commonly affected are the knees, ankles, and wrists.

Gastrointestinal symptoms: Behcet’s disease can cause abdominal pain, diarrhea, and bleeding. It can also lead to ulcerations and perforations in the digestive tract.

Neurological symptoms: Behcet’s disease can cause a range of neurological symptoms, including headache, dizziness, and seizures.

The symptoms of Behcet’s disease can come and go and may vary in severity over time. The disease may also progress slowly over time, leading to a worsening of symptoms and potential complications. A thorough medical history and physical examination, as well as appropriate laboratory testing, are necessary to make an accurate diagnosis of Behcet’s disease.

Physical examination

A physical examination is an important part of the diagnostic process for Behcet’s disease. The examination may reveal various signs and symptoms, depending on the specific organs and systems affected by the disease. Some of the physical findings that may be seen in Behcet’s disease include:

  • Oral ulcers: round or oval ulcers on the tongue, inside of the cheeks, or on the lips.
  • Genital ulcers: painful ulcers on the penis, vulva, or vagina.
  • Skin lesions: red or purple, raised or flat lesions on the skin, including erythema nodosum, papulopustular lesions, and nodules.
  • Eye inflammation: redness, pain, and sensitivity to light, along with inflammation in the eye that can be seen on an eye exam.
  • Joint inflammation: swelling, redness, and tenderness in the joints, most commonly in the knees, ankles, and wrists.
  • Gastrointestinal symptoms: abdominal tenderness, bloating, and diarrhea, as well as signs of inflammation on imaging tests.
  • Neurological symptoms: signs of neurological involvement, such as headache, dizziness, and altered mental status.

Differential diagnosis

The clinical manifestations of Behcet’s disease can vary widely, and as a result, the differential diagnosis may include a range of other conditions that share similar symptoms. Some of the conditions that may be considered in the differential diagnosis of Behcet’s disease include:

  • Oral and genital ulcers: recurrent aphthous stomatitis, herpes simplex virus infection, syphilis, Crohn’s disease, and other inflammatory bowel diseases
  • Skin lesions: erythema nodosum, erythema multiforme, cutaneous vasculitis, lupus erythematosus, and pyoderma gangrenosum
  • Eye inflammation: uveitis, scleritis, acute angle-closure glaucoma, and herpes simplex virus infection
  • Joint pain and inflammation: rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and systemic lupus erythematosus
  • Neurological symptoms: multiple sclerosis, meningitis, and stroke.

It is important to note that Behcet’s disease is a diagnosis of exclusion, meaning that other potential causes of the symptoms must be ruled out before a definitive diagnosis can be made. A thorough medical history, physical examination, and laboratory testing may be necessary to make an accurate diagnosis and to differentiate Behcet’s disease from other conditions with similar symptoms.

Treatment of Behcet’s disease aims to control the symptoms and prevent complications associated with the disease. The treatment plan may vary depending on the severity and location of the symptoms and may include medications and lifestyle modifications. Some treatment options for Behcet’s disease include:

Nonsteroidal anti-inflammatory drugs (NSAIDs): These medications can be effective in treating joint pain and inflammation associated with Behcet’s disease.

Corticosteroids: Steroid medications can be effective in reducing inflammation associated with Behcet’s disease, especially in more severe cases.

Immunomodulatory drugs: Medications that suppress the immune system, such as azathioprine, methotrexate, and cyclosporine, can be effective in treating severe cases of Behcet’s disease.

Biologic agents: These medications target specific parts of the immune system and can be effective in treating severe or refractory Behcet’s disease.

Colchicine: This medication can be effective in reducing the frequency and severity of oral and genital ulcers associated with Behcet’s disease.

Lifestyle modifications: Avoiding triggers that may exacerbate symptoms, such as stress, certain foods, and smoking, can help manage symptoms of Behcet’s disease.

https://www.ncbi.nlm.nih.gov/books/NBK470257/