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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 141: Adamantiades–Behçet Disease > Flashcards

141: Adamantiades–Behçet Disease Flashcards

1
Q

What are the common clinical manifestations of Behcet Disease?

A

The common clinical manifestations of Behcet Disease include:
- Oral aphthous ulcers
- Genital ulcers
- Papulopustules
- Erythema nodosum-like lesions
- Uveitis
- Arthropathy

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2
Q

What is the genetic association observed in Behcet Disease?

A

Behcet Disease has several genetic associations, including:
- HLAB 51 association observed in Japan, the Middle East, and Mediterranean countries, linked to more severe prognosis and ocular involvement.
- Familial occurrences are more common in Korea (15%).
- Altered peptide presentation is vital to the disease process, involving NK or other cell interactions.
- Polymorphisms in genes such as IL23R may contribute to disease susceptibility and severity.

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3
Q

What is the epidemiological pattern of Behcet Disease?

A

Behcet Disease occurs worldwide, with the following epidemiological patterns:
- Endemic in Eastern and Central Asian and Eastern Mediterranean countries (Silk Road).
- Annual incidence is low.
- Most often affects patients in their 20s and 30s.
- Both genders are equally affected overall, but male predominance is observed in Arab populations, while female predominance is noted in Korea, China, some Northern European countries, and the United States.

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4
Q

What is the chronic course and prognosis of Behcet Disease?

A

Behcet Disease is characterized by a chronic, relapsing, progressive course with a potentially poor prognosis, especially in males with systemic presenting signs. The mortality rate is reported to be between 0-6%.

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5
Q

What are the proposed environmental and genetic factors in the etiology of Behcet Disease?

A

The etiology of Behcet Disease remains unknown, but it is believed to be influenced by:
- Genetic factors: Familial occurrences and specific genetic associations (e.g., HLAB 51).
- Environmental factors: The endemic occurrence along the historical Silk Road suggests a link to the migration of old nomadic tribes.

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6
Q

What type of disease is Behcet Disease classified as?

A

A multisystem inflammatory disease classified as systemic vasculitis involving all types and sizes of blood vessels.

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7
Q

What are the common signs of Behcet Disease?

A

Common signs include:
- Oral aphthous ulcers
- Genital ulcers
- Papulopustules
- Erythema nodosum-like lesions
- Uveitis
- Arthropathy

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8
Q

What is the typical course of Behcet Disease?

A

Chronic, relapsing, progressive course with potentially poor prognosis.

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9
Q

What is the annual incidence of Behcet Disease?

A

The annual incidence is low.

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10
Q

In which populations is male predominance observed for Behcet Disease?

A

Male predominance is observed in Arab populations.

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11
Q

What genetic association is linked to Behcet Disease?

A

HLAB 51 association is observed, particularly in Japan, the Middle East, and Mediterranean countries.

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12
Q

What is the significance of the Bw4 epitope in Behcet Disease?

A

The Bw4 epitope contributes to the severity of the disease and is crucial for antigen binding and NK cell interactions.

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13
Q

What is the familial occurrence rate of Behcet Disease in Korea?

A

Familial occurrences occur more often in Korea, with a rate of 15%.

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14
Q

What environmental factor is suggested to trigger Behcet Disease?

A

A probable environmental triggering factor is suggested, alongside genetic predisposition.

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15
Q

What is the relationship between Behcet Disease and the Silk Road?

A

The endemic occurrence along the historical Silk Road supports the hypothesis that the disease followed the migration of old nomadic tribes.

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16
Q

What are the common clinical manifestations of Adamantiades-Behcet Disease?

A

The most common signs include:
- Oral aphthous ulcers
- Genital ulcers
- Papulopustules
- Erythema nodosum-like lesions
- Uveitis
- Arthropathy

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17
Q

How does the epidemiology of Adamantiades-Behcet Disease vary by gender and geography?

A

The disease occurs worldwide but is endemic in the Eastern and Central Asian and Eastern Mediterranean countries.

  • Gender Distribution:
    • Both genders are equally affected overall.
    • Male predominance is observed in Arab populations.
    • Female predominance is evident in Korea, China, and some Northern European countries.
  • Age of Onset:
    • Most often affects patients in their 20s and 30s.
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18
Q

What role do genetic factors play in the pathogenesis of Adamantiades-Behcet Disease?

A

Genetic factors associated with Adamantiades-Behcet Disease include:
- HLA-B51 association: Observed in Japan, the Middle East, and Mediterranean countries, linked to more severe prognosis and ocular involvement.
- Polymorphisms in genes: May contribute to disease susceptibility and severity (e.g., IL23R in Chinese Han population).
- Other gene associations: New associations with ERAP1, CCR1-CCR3, KLRC4, and STAT4 genes have been reported.

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19
Q

What are the potential environmental and genetic triggers for Adamantiades-Behcet Disease?

A

The disease is thought to be genetically determined with a probable environmental triggering factor. Key points include:
- Familial occurrences: More common in Korea (15%).
- Earlier disease onset: Observed in children compared to their parents.
- Endemic occurrence: Along the historical Silk Road, suggesting migration patterns of nomadic tribes may play a role.

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20
Q

What is the significance of the HLA-B51 association in Adamantiades-Behcet Disease?

A

The HLA-B51 association is significant because:
- It is linked to more severe prognosis and ocular involvement.
- It involves altered peptide presentation, which is vital to the disease process through interactions with NK cells and other immune mechanisms.
- The Bw4 epitope shared among residues is crucial for antigen binding and contributes to the severity of the disease.

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21
Q

What are the most common signs of Adamantiades-Behcet Disease?

A

The most common signs include:
- Oral aphthous ulcers
- Genital ulcers
- Papulopustules
- Erythema nodosum-like lesions
- Uveitis
- Arthropathy

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22
Q

What is the epidemiology of Adamantiades-Behcet Disease regarding gender and age?

A

The disease affects both genders equally overall; however:
- Male predominance is observed in Arab populations.
- Female predominance is evident in Korea, China, some Northern European countries, and the United States.
- Most often affects patients in their 20s and 30s.

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23
Q

What is the significance of HLA-B51 in Adamantiades-Behcet Disease?

A

HLA-B51 is associated with:
- More severe prognosis
- Ocular involvement
- Altered peptide presentation, which is vital to the disease process through interactions with NK cells and other immune responses.

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24
Q

What are the potential environmental and genetic factors contributing to Adamantiades-Behcet Disease?

A

The disease is thought to be genetically determined with a probable environmental triggering factor. Key points include:
- Familial occurrences are more common in Korea.
- Earlier disease onset in children compared to their parents.
- Genetic associations with various genes, including HLA-B51, have been identified.

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25
Q

What is the classification of Adamantiades-Behcet Disease?

A

Adamantiades-Behcet Disease is classified as a multisystem inflammatory disease of unknown etiology, specifically categorized as systemic vasculitis involving all types and sizes of blood vessels.

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26
Q

What is the role of Streptococcus sanguis in the initiation of disease in patients?

A

Streptococcus sanguis dominates the flora of the oral mucosa and is considered the most relevant strain as a provoking factor for the initiation of disease. It produces IgA protease and has been proposed as an explanation for chronic infection leading to disease initiation.

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27
Q

What are the major immunologic mechanisms involved in the disease process?

A

The major immunologic mechanisms include:
1. Autoimmune mechanisms: Characterized by immune-mediated occlusive vasculitis and pathergy reaction, predominantly Th1 mediated by IL-12, with high levels of IFN-γ, IL-2, and TNF-α.
2. Heat shock proteins: 60kDa HSP identified by T cell mapping.
3. Cytokine mediators: IL-8 is a sensitive marker of disease activity, along with IL-1, IL-12, IL-17, IL-23, and TNF-α.
4. Endothelial cells: The endothelium is the primary target, with increased plasma endothelin-1 and thrombomodulin in patients with active disease, indicating damage to endothelial cells.

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28
Q

What are the clinical findings associated with mucocutaneous lesions in the disease?

A

The clinical findings associated with mucocutaneous lesions include:
- Recurrent oral aphthous and genital ulcers: These are the most frequently observed mucosal manifestations.
- Oral aphthous ulcers: Presenting sign in 80% of cases, characterized by multiple, painful ulcers (1-3mm) with a fibrin-coated base and surrounding erythema. They typically heal spontaneously within 4 days to 1 month without scarring (92%).

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29
Q

Are infectious agents considered contagious in this context?

A

No, as no horizontal transmission has ever been reported.

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30
Q

What is the most relevant bacterial agent provoking disease initiation?

A

Streptococci sanguis.

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31
Q

What immune response is predominantly involved in the disease process?

A

Th1 mediated by IL-12, high levels of IFN-γ, IL-2, TNF-α.

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32
Q

What is a major microscopic finding in active disease?

A

Immune-mediated occlusive vasculitis.

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33
Q

What cytokine is a sensitive marker of disease activity?

A

IL-8.

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34
Q

What are the most frequently observed mucosal manifestations?

A

Recurrent oral aphthous and genital ulcers.

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35
Q

What is the typical healing time for oral aphthous ulcers?

A

Spontaneous healing in 4 days to 1 month.

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36
Q

What percentage of oral ulcers heal without scarring?

A

92%.

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37
Q

What is the role of heat shock proteins in the disease?

A

Identified by T cell mapping, they may be involved in the immune response.

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38
Q

What alterations are involved in the epigenetics of the disease?

A

Alterations in methylation level, histone modifications, and gene regulation.

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39
Q

What is the role of Streptococcus sanguis in the initiation of disease, and how does it contribute to chronic infection?

A

Streptococcus sanguis dominates the oral mucosa flora and is the most relevant strain for initiating disease. It produces IgA protease, which may explain chronic infection leading to disease initiation.

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40
Q

Describe the autoimmune mechanisms involved in the pathogenesis of the disease as mentioned in the content.

A

The autoimmune mechanisms include:
1. Immune-mediated occlusive vasculitis at most active disease sites.
2. Pathergy reaction: Rapid accumulation of neutrophils, followed by T lymphocytes and monocytes at needle prick sites.
3. Cytokine involvement: Predominantly Th1 mediated by IL-12, with high levels of IFN-γ, IL-2, and TNF-α.

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41
Q

What are the clinical implications of increased plasma endothelin-1 in patients with active disease?

A

Increased plasma endothelin-1 signifies vasoconstriction, which is a direct result of elevated synthesis by injured vascular endothelial cells.

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42
Q

How do mucocutaneous lesions manifest in patients, and what is the significance of the Mucocutaneous Activity Index?

A

Mucocutaneous lesions often present as recurrent oral aphthous and genital ulcers, which are the most frequently observed mucosal manifestations.

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43
Q

What is the significance of heat shock proteins in the context of the disease, and how are they identified?

A

Heat shock proteins, specifically the 60kDa HSP, are identified by T cell mapping and play a role in the immune response.

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44
Q

What are the main infectious agents associated with the initiation of disease in patients, and how do they contribute?

A
  1. Viral agents: HSV 1 DNA is noted but not considered contagious.
  2. Bacterial agents:
    • Streptococcus sanguis: Dominates oral flora, produces IgA protease, and is linked to chronic infection and disease initiation.
45
Q

What immunologic mechanisms are involved in the pathogenesis of the disease?

A
  1. Immune-mediated occlusive vasculitis is a major finding.
  2. Pathergy reaction: Rapid accumulation of neutrophils and T lymphocytes at needle prick sites.
46
Q

What role do endothelial cells play in the disease process?

A

Endothelial cells are primary targets in the disease, with increased plasma endothelin-1 indicating vasoconstriction due to elevated synthesis by injured endothelial cells.

47
Q

What are the clinical findings associated with mucocutaneous lesions in this disease?

A

Recurrent oral aphthous and genital ulcers are the most common mucosal manifestations.

48
Q

What are the characteristics of genital ulcers in this condition?

A

Genital ulcers involve the penis, scrotum, vagina, labia, urethra, anal, perineal, and inguinal regions. They heal with a characteristic scar in 64% - 88% of cases and tend to persist longer.

49
Q

What types of skin lesions are associated with this condition?

A

The skin lesions include pustular vasculitic lesions, EN-like lesions, Sweet-like lesions, PG-like lesions, and palpable lesions.

50
Q

What types of skin lesions are associated with this condition?

A

The skin lesions include pustular vasculitic lesions, EN-like lesions, Sweet-like lesions, PG-like lesions, and palpable purpuric lesions of necrotizing venulitis, all characterized by a neutrophilic vascular reaction.

51
Q

What is the major cause of morbidity related to ocular involvement in this condition?

A

The major cause of morbidity is posterior uveitis (retinal vasculitis), which can lead to blindness. Other ocular lesions include anterior uveitis and hypopyon, with complications such as cataract and glaucoma.

52
Q

Describe the characteristics of arthritis associated with this condition.

A

The arthritis is non-erosive, asymmetric, sterile, sero-negative, and oligoarthritis. It may start in one knee or ankle, leading to migratory monoarthritis and potentially affecting multiple joints.

53
Q

What are the systemic vascular involvements seen in this condition?

A

Systemic vascular involvement is rare, typically presenting as thromboses or, less often, aneurysms. Pulmonary artery aneurysms are a principal feature, leading to coughing and hemoptysis, while large vein thrombosis can be fatal.

54
Q

What renal manifestations are associated with this condition?

A

Renal manifestations include minimal change disease, proliferative glomerulonephritis, and rapidly progressive crescentic glomerulonephritis, often due to immune complex deposition.

55
Q

What are the neurologic manifestations associated with this condition?

A

Neurologic manifestations can include severe headache, meningoencephalitis, cerebral venous sinus thrombosis, and various symptoms such as gait disturbance, dysarthria, and psychiatric symptoms like depression and memory impairment.

56
Q

What are the potential fatal complications of systemic vascular involvement?

A

Large vein thrombosis or large artery aneurysms.

57
Q

What cardiac issues can arise from this condition?

A

Myocarditis, coronary arteritis, endocarditis, and valvular disease.

58
Q

What are the gastrointestinal complaints associated with this condition?

A

Perforation and peritonitis, with an acute exacerbating course in the ileocolonic area.

59
Q

What psychiatric symptoms may be present?

A

Depression, insomnia, and memory impairment.

60
Q

What are the key ocular manifestations associated with systemic lesions and their potential consequences?

A
  • Major cause of morbidity: Posterior uveitis (retinal vasculitis) can lead to blindness.
  • Other lesions include anterior uveitis and hypopyon.
  • Complications: cataract, glaucoma, neovascular lesions.
  • Severe vitreous involvement can cause vision loss due to chronic cystoid macular edema and vasculitic involvement of the optic nerve.
  • Recurrent vasculitic changes may lead to ischemic optic nerve atrophy.
61
Q

How does systemic vascular involvement present in this condition, and what are the potential risks?

A
  • Arterial involvement is rare, typically presenting as thromboses rather than aneurysms.
  • Aneurysms may develop in large arteries due to vasculitis affecting the vasa vasorum.
  • Pulmonary artery aneurysms are significant, leading to coughing and hemoptysis.
  • Large vein thrombosis (e.g., inferior vena cava) or large artery aneurysms can be potentially fatal.
62
Q

What are the clinical implications of renal manifestations in this disease?

A
  • Minimal change disease can progress to proliferative glomerulonephritis, leading to rapidly progressive crescentic glomerulonephritis.
  • This is due to immune complex deposition.
  • Clinical biomarkers for prognosis include:
    • Carotid plaques
    • Pulse wave velocity
    • Flow mediated dilation
63
Q

What are the common neurological manifestations and their potential impact on patients?

A
  • Neurological manifestations often present as severe headache.
  • Other symptoms include:
    • Meningoencephalitis
    • Cerebral venous sinus thrombosis
    • Cranial nerve palsies
    • Brainstem lesions
    • Pyramidal or extrapyramidal lesions
  • Additional symptoms: gait disturbance, dysarthria, vertigo, diplopia, hyperreflexia, seizures, hemiplegia, ataxia, positive Babinski reflex.
  • Psychiatric symptoms may include depression, insomnia, and memory impairment.
  • Prognosis: Generally poor with a progressive course and relapses after treatment.
64
Q

What are the characteristic histopathological features of Behcet’s disease?

A

The characteristic histopathological features of Behcet’s disease include:
- Vasculitis and thrombosis
- Early lesions: Neutrophilic vascular reaction with endothelial swelling, extravasation of erythrocytes, and leukocytoclasia
- Older lesions: Lymphocytic vasculitis
- Fully developed LCV: Fibrinoid necrosis of vessel walls
- Predominant finding: Neutrophilic vascular reaction

65
Q

What is the significance of a positive pathergy test in Behcet’s disease?

A

A positive pathergy test indicates hyperreactivity and is characterized by:
- An erythematous papule (>2 mm) or pustule at the site of skin needle prick or after intracutaneous injection of isotonic saline within 48 hours.
- Although a positive reaction is a sign of the disease, it is not pathognomonic for Behcet’s disease.

66
Q

What are the risk factors for the development of systemic vessel involvement in Behcet’s disease?

A

The risk factors for the development of systemic vessel involvement in Behcet’s disease include:
1. Superficial thrombophlebitis
2. Ocular lesions
3. Male gender

67
Q

What are the major life-threatening complications associated with Behcet’s disease?

A

The major life-threatening complications of Behcet’s disease include:
1. CNS and pulmonary large vessel involvement
2. GI perforation

68
Q

What treatment options are available for recurrent aphthae in Behcet’s disease?

A

Treatment options for recurrent aphthae in Behcet’s disease include:
1. Mild diet
2. Avoidance of irritating agents
3. Potent topical glucocorticoids
4. Local anesthetics
5. Topical hyaluronic acid 0.2% gel applied BID over 30 days

69
Q

What are the characteristic histopathological features of Behcet’s disease?

A

Vasculitis and thrombosis, with early lesions showing neutrophilic vascular reaction and older lesions showing lymphocytic vasculitis.

  • Vasculitis and thrombosis
  • Early lesions: Neutrophilic vascular reaction with endothelial swelling, extravasation of erythrocytes, and leukocytoclasia
  • Older lesions: Lymphocytic vasculitis
  • Fully developed LCV: Fibrinoid necrosis of vessel walls
  • Predominant finding: Neutrophilic vascular reaction
70
Q

What is the pathergy test and what does a positive result indicate?

A

The pathergy test involves an erythematous papule or pustule at the site of a skin needle prick, indicating hyperreactivity, but it is not pathognomonic for the disease.

  • A positive test results in:
    • An erythematous papule (>2 mm) or pustule at the site of skin needle prick or after an intracutaneous injection of saline.
    • It is performed by placing a skin prick at a 45° angle on the volar arm without prior disinfection.
71
Q

What are the common clinical manifestations of Behcet’s disease?

A

Mucocutaneous and joint manifestations usually occur first, followed by ocular and vascular involvement.

72
Q

What are the major life-threatening complications associated with Behcet’s disease?

A

CNS and pulmonary large vessel involvement, and gastrointestinal perforation.

73
Q

What risk factors are associated with the development of systemic vessel involvement in Behcet’s disease?

A

Superficial thrombophlebitis, ocular lesions, and male gender.

74
Q

What is the significance of HLA B51 in Behcet’s disease?

A

HLA B51 is a marker associated with severe prognosis and risk factors for complications.

75
Q

What treatment options are available for recurrent aphthae in Behcet’s disease?

A

Mild diet, avoidance of irritating agents, potent topical glucocorticoids, local anesthetics, and topical hyaluronic acid gel.

  • Topical hyaluronic acid 0.2% gel applied BID over 30 days.
76
Q

What are the common neurological findings in Behcet’s disease?

A

Cranial MRI may show hypodense or atrophic changes in the brain, and EEG may show diffuse alpha waves.

77
Q

What are the leading causes of morbidity in Behcet’s disease?

A

Ophthalmic and neurologic sequelae, followed by severe vascular and gastrointestinal manifestations.

78
Q

How do the clinical manifestations of Behcet’s disease differ between males and females?

A

The clinical manifestations of Behcet’s disease differ as follows:

Gender | Common Manifestations |
|——–|———————-|
| Male | - Ocular involvement |
| | - Vascular involvement|
| | - Superficial and deep venous thrombosis |
| | - Cardiac involvement |
| | - Folliculitis |
| | - Papulopustular lesions |
| Female | - Genital ulcers |
| | - Erythema nodosum |
| | - Joint involvement |

79
Q

What are the treatment options for painful lesions in patients with Behcet’s disease?

A
  1. Antiseptic creams for 1 week
  2. If painful, use topical anesthetics
  3. If recalcitrant, consider intralesional triamcinolone acetonide (0.1 - 0.5ml/lesion)
80
Q

What are the systemic treatment candidates for Behcet’s disease?

A
  1. Patients resistant to topical treatment
  2. Patients with systemic involvement
  3. Patients with markers for poor prognosis
81
Q

What are the treatment options for panuveitis and cystoid macular edema in Behcet’s disease?

A
  1. Intravitreal injection of triamcinolone (TA 4 mg)
  2. Cyclosporine A
82
Q

What medications can be combined with oral and IV prednisolone in the treatment of Behcet’s disease?

A

Oral and IV prednisolone can be combined with:
- Other immunosuppressants
- Colchicine
- Dapsone
- Sulfasalazine
- IFN - α

83
Q

Why is prednisolone considered a suitable medication during pregnancy for Behcet’s disease?

A

Prednisolone is one of the few medications that can be used during pregnancy without significant risk to the fetus.

84
Q

What is the recommended treatment for lesions if they are painful?

A

Topical anesthetics.

85
Q

What is the treatment for recalcitrant lesions?

A

Intralesional triamcinolone acetonide (0.1 - 0.5ml/lesion).

86
Q

What is the treatment for panuveitis and cystoid macular edema?

A

Intravitreal injection of triamcinolone acetonide (TA 4 mg) and Cyclosporine A.

87
Q

Who are candidates for systemic treatment?

A

Patients resistant to topical treatment, patients with systemic involvement, and patients with markers for poor prognosis.

88
Q

What are the criteria for candidates requiring systemic treatment in Behçet’s disease?

A
  1. Patients resistant to topical treatment
  2. Patients with systemic involvement
  3. Patients with markers for poor prognosis
89
Q

What are the risk factors for development of vision loss in Behcet disease?

A

Risk factors for vision loss include:
1. Uveitis - Inflammation of the uvea can lead to vision impairment.
2. Retinal involvement - Damage to the retina can cause significant vision loss.
3. Systemic involvement - Patients with systemic manifestations may have a higher risk of ocular complications.
4. Delayed treatment - Late intervention can worsen outcomes.

90
Q

What are the major causes of morbidity in Behcet disease?

A

Major causes of morbidity include:
1. Recurrent oral ulcers - Painful and debilitating.
2. Skin lesions - Such as erythema nodosum or pseudofolliculitis.
3. Ocular complications - Including uveitis and retinal damage.
4. Joint pain - Arthritis or arthralgia can significantly affect quality of life.

91
Q

What are the major causes of mortality in Behcet disease?

A

Major causes of mortality include:
1. Vascular complications - Such as thrombosis or aneurysms.
2. Neurological involvement - Central nervous system complications can be life-threatening.
3. Severe systemic disease - Multi-organ failure due to systemic involvement.
4. Infections - Secondary to immunosuppressive treatments.

92
Q

What is the most common presenting sign of Behcet disease?

A

The most common presenting sign of Behcet disease is recurrent oral aphthous ulcers. These painful sores are often the first symptom that patients experience.

93
Q

What are the markers of severe prognosis in Behcet disease?

A

Markers of severe prognosis include:
1. Early age of onset - Younger patients tend to have a more aggressive disease course.
2. Male gender - Males often experience more severe manifestations.
3. Systemic involvement - Presence of systemic symptoms indicates a worse prognosis.
4. Familial history - A family history of Behcet disease can suggest a more severe form.

94
Q

What are the International Criteria for Behcet Disease?

A

The International Criteria for Behcet Disease include:
1. Recurrent oral ulcers
2. Recurrent genital ulcers
3. Eye lesions - Uveitis or retinal vasculitis.
4. Skin lesions - Such as pseudofolliculitis or erythema nodosum.
5. Positive pathergy test - A skin reaction to needle prick.

Diagnosis typically requires the presence of at least three of these criteria.

95
Q

What is a positive pathergy reaction?

A

A positive pathergy reaction is characterized by:
- Skin reaction - Development of a papule or pustule at the site of a needle prick within 24-48 hours.
- Clinical significance - It is considered a diagnostic criterion for Behcet disease and indicates heightened skin reactivity.

96
Q

What often occurs rapidly after discontinuing cyclosporine A, IFN α, dapsone, or infliximab?

A

Rapid relapse of symptoms.

97
Q

What should patients with severe or progressive recurrent aphthous stomatitis be followed up for?

A

Potential candidates for Adamantiades - Behcet disease.

98
Q

Why should male patients with systemic involvement be treated systematically?

A

Due to the poor prognosis associated with their condition.

99
Q

What is recommended for patients with suspected Behcet disease?

A

They should be referred early for specialist advice.

100
Q

What are the risk factors for systemic vessel involvement in Behcet Disease?

A

Risk factors for systemic vessel involvement include:
- Male patients
- Early age of onset
- Presence of severe symptoms
- Family history of Behcet Disease

101
Q

What are the major causes of morbidity in Behcet Disease?

A

Major causes of morbidity include:
- Ocular involvement leading to vision loss
- Recurrent oral and genital ulcers
- Skin lesions
- Vascular complications

102
Q

What are the major causes of mortality in Behcet Disease?

A

Major causes of mortality include:
- Vascular complications (e.g., thrombosis)
- CNS involvement (e.g., meningoencephalitis)
- Severe systemic involvement

103
Q

What is the most common presenting sign of Behcet Disease?

A

The most common presenting sign is recurrent oral aphthous ulcers.

104
Q

What are the markers of severe prognosis in Behcet Disease?

A

Markers of severe prognosis include:
- Early age of onset
- Male gender
- Presence of systemic involvement
- Severe ocular manifestations

105
Q

What are the International Criteria for Behcet Disease?

A

The International Criteria for Behcet Disease include:
- Ocular lesions (recurrent) - 2 points
- Oral aphthosis (recurrent) - 2 points
- Genital aphthosis (recurrent) - 2 points
- Skin lesions (recurrent) - 1 point
- CNS involvement - 1 point
- Vascular manifestations - 1 point
- Positive pathergy test - 1 point

106
Q

What is the positive pathergy reaction in the context of Behcet Disease?

A

The positive pathergy reaction is characterized by:
- A papule or pustule developing at the site of a minor skin prick or injury within 24-48 hours, indicating an abnormal immune response.

107
Q

What are the risk factors for the development of vision loss in Behcet Disease?

A

Risk factors for vision loss in Behcet Disease include:
- Ocular lesions (recurrent)
- Systemic involvement
- Early age of onset
- Male gender

108
Q

What are the recommended topical treatments for oral aphthous ulcers?

A

The recommended topical treatments include:
1. Causitic solutions (e.g., silver nitrate)
2. Topical anesthetics (e.g., lidocaine)
3. Corticosteroids (e.g., triamcinolone)
4. Anesthetics (e.g., lidocaine)
5. 5-Aminosalicylic acid
6. Cyclosporine
7. Sucralfate suspension
8. Glycyrrhizin

109
Q

What systemic treatments are studied for Behçet’s disease and their indications?

A

The studied systemic treatments for Behçet’s disease include:
| Drug | Dose | Indication |
|——|——|———–|
| Methylprednisolone | 40 mg every week | Erythema nodosum (not ocular lesions) |
| Colchicine | 1-2 mg daily | Oral aphthous ulcers, folliculitis |
| Dapsone | 100 mg daily | Genital ulcers, skin lesions |
| Azathioprine | 2-3 mg/kg daily | Ocular disease, bone marrow depression |
| Interferon-alpha 2a | 6 x 10^6 units 3 times a week | Genital ulcers, skin lesions |
| Thalidomide | 100 mg daily | Oral ulcers, skin lesions |
| Cyclophosphamide | 10 mg/kg daily | Ocular manifestations, skin lesions |
| Adalimumab | 80 mg subcutaneously | Intermediate uveitis, skin lesions |
| Etanercept | 25 mg twice a week | Ocular arthritis, nodular lesions |

Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) (48 decks)

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