Behçet disease: Difference between revisions
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+ | == Clinical Manifestations == |
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+ | |||
+ | * Skin and mucosal |
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+ | ** '''Oral ulcers''' (98%), which are usually painful, recurrent, and can involve the soft and hard palate, buccal mucosa, tongue, gingiva, lips, and tonsils, and usually heal without scarring |
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+ | ** '''Genital ulcers''' (65%): recurrent, mostly heal ''with'' scarring, most commonly on scrotum in men and vulva or vagina in women |
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+ | ** Orchiepididymitis (6%) |
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+ | ** '''Skin lesion''' (75%), including [[pseudofolliculitis]], acne-like papulopustular lesions, [[erythema nodosum]], positive [[pathergy test]] |
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+ | * '''Joints''' (50%) |
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+ | ** [[Oligoarticular arthritis]]: primarily involving knees, ankles, wrists, and elbows; non-erosive and non-deforming, and more common in patients with acneiform skin lesions |
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+ | ** Can be a [[polyarticular arthritis]] as well |
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+ | ** Usually excludes [[sacroiliitis]] |
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+ | ** [[Enthesopathy]] |
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+ | * '''Ocular''' (50%): [[hypopyon]], non-granulomatous [[panuveitis]], [[retinal vasculitis]] |
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+ | * CNS involvement (10-30%): mesodiencephalic and brain-stem inflammation, encephalitis, meningitis, myelitis, neurocognitive dysfunction, cerebral-vein thrombosis |
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+ | * Cardiovascular |
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+ | ** Arterial (2-18%): arterial aneurysms, arterial stenosis, arterial thrombosis |
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+ | ** Cardiac (6%): valvulitis, myopericarditis, coronary arteritis |
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+ | ** Venous (15-40%): [[DVT]], [[superficial thrombophlebitis]] |
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+ | * Gastrointestinal (0-20%): mucosal ulcers, abdominal pain, hemorrhage, perforation |
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+ | |||
== Diagnostic Criteria == |
== Diagnostic Criteria == |
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+ | === ICBD 2014 === |
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⚫ | |||
+ | |||
+ | * Criteria: |
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+ | ** Recurrent oral ulcers (2 points) |
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+ | ** Genital ulcers (2 points) |
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+ | ** Ocular lesions (2 points) |
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+ | ** Skin lesions (1 point) |
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+ | ** Vascular manifestations (1 point) |
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+ | ** Neurologic manifestations (1 point) |
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+ | ** Positive pathergy test (optional) (1 point) |
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+ | * Interpretation: diagnosis based on ≥4 points |
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+ | ** Sensitivity 95%, specificity 92% |
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+ | |||
+ | === ISG 1990 === |
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⚫ | |||
* Any two of the following: |
* Any two of the following: |
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** Recurrent genital ulcerations |
** Recurrent genital ulcerations |
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** Skin lesions ([[erythema nodosum]], [[pseudofolliculitis]], [[Papulopustular lesion|papulopustular lesions]], [[Aceiform nodule|aceiform nodules]]) in adult patients not treated with corticosteroids |
** Skin lesions ([[erythema nodosum]], [[pseudofolliculitis]], [[Papulopustular lesion|papulopustular lesions]], [[Aceiform nodule|aceiform nodules]]) in adult patients not treated with corticosteroids |
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** Positive [[pathergy test]] read by a physician at 24 to 48 hours |
** Positive [[pathergy test]] read by a physician at 24 to 48 hours |
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+ | * Sensitivity 95%, specificity 96% |
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+ | |||
+ | == Management == |
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+ | |||
+ | * Isolated skin and mucosal ulcers: tropical treatments for active ulcers, with [[colchicine]] as preventative treatment |
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+ | * Refractory or widespread lesions or ulcers: prednisone 15 mg daily for 1 week, followed by a taper over several weeks |
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+ | * Other options for more advanced presentations include systemic glucocorticoids, [[azathioprine]], [[TNF-α inhibitors]], |
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+ | |||
+ | == Further Reading == |
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+ | |||
+ | * Behçet Syndrome. ''NEJM'' 2024;390(7):640-651. doi: [https://doi.org/10.1056/NEJMra2305712 10.1056/NEJMra2305712] |
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[[Category:Dermatology]] |
[[Category:Dermatology]] |
Latest revision as of 12:28, 13 March 2024
Clinical Manifestations
- Skin and mucosal
- Oral ulcers (98%), which are usually painful, recurrent, and can involve the soft and hard palate, buccal mucosa, tongue, gingiva, lips, and tonsils, and usually heal without scarring
- Genital ulcers (65%): recurrent, mostly heal with scarring, most commonly on scrotum in men and vulva or vagina in women
- Orchiepididymitis (6%)
- Skin lesion (75%), including pseudofolliculitis, acne-like papulopustular lesions, erythema nodosum, positive pathergy test
- Joints (50%)
- Oligoarticular arthritis: primarily involving knees, ankles, wrists, and elbows; non-erosive and non-deforming, and more common in patients with acneiform skin lesions
- Can be a polyarticular arthritis as well
- Usually excludes sacroiliitis
- Enthesopathy
- Ocular (50%): hypopyon, non-granulomatous panuveitis, retinal vasculitis
- CNS involvement (10-30%): mesodiencephalic and brain-stem inflammation, encephalitis, meningitis, myelitis, neurocognitive dysfunction, cerebral-vein thrombosis
- Cardiovascular
- Arterial (2-18%): arterial aneurysms, arterial stenosis, arterial thrombosis
- Cardiac (6%): valvulitis, myopericarditis, coronary arteritis
- Venous (15-40%): DVT, superficial thrombophlebitis
- Gastrointestinal (0-20%): mucosal ulcers, abdominal pain, hemorrhage, perforation
Diagnostic Criteria
ICBD 2014
- Criteria:
- Recurrent oral ulcers (2 points)
- Genital ulcers (2 points)
- Ocular lesions (2 points)
- Skin lesions (1 point)
- Vascular manifestations (1 point)
- Neurologic manifestations (1 point)
- Positive pathergy test (optional) (1 point)
- Interpretation: diagnosis based on ≥4 points
- Sensitivity 95%, specificity 92%
ISG 1990
- Recurrent oral ulcerations (aphthous or herpetiform) at least three times in one year, plus
- Any two of the following:
- Recurrent genital ulcerations
- Eye lesions 9uveitis or retinal vasculitis) observed by an ophthalmologist
- Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, aceiform nodules) in adult patients not treated with corticosteroids
- Positive pathergy test read by a physician at 24 to 48 hours
- Sensitivity 95%, specificity 96%
Management
- Isolated skin and mucosal ulcers: tropical treatments for active ulcers, with colchicine as preventative treatment
- Refractory or widespread lesions or ulcers: prednisone 15 mg daily for 1 week, followed by a taper over several weeks
- Other options for more advanced presentations include systemic glucocorticoids, azathioprine, TNF-α inhibitors,
Further Reading
- Behçet Syndrome. NEJM 2024;390(7):640-651. doi: 10.1056/NEJMra2305712