Behcet's disease: Difference between revisions
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* Age 20-30s MC | * Age 20-30s MC | ||
* Turkey, Japan, Middle East | * Turkey, Japan, Middle East | ||
* Triad: | * Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis | ||
* Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis | * Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis | ||
* Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon | * Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon | ||
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* GI: Ulcers, obstruction, ileocecal perforation | * GI: Ulcers, obstruction, ileocecal perforation | ||
* Inflammatory oligoarthritis | * Inflammatory oligoarthritis | ||
* Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis | |||
* Cardiac: Myocarditis, endocarditis, pericarditis | |||
* Renal: Glomerulonephritis, amyloidosis | |||
* Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
* HSV, lichen planus, pemphigus vulgaris, pemphigoid, IVD, SJS, celiac dz, SLE, auto-inflammatory disorders | * HSV, lichen planus, pemphigus vulgaris, pemphigoid, IVD, SJS, celiac dz, SLE, auto-inflammatory disorders | ||
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==Classification== | ==Classification== | ||
* International Study Group Criteria | * International Study Group Criteria | ||
* Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other | * Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases: | ||
** Recurrent genital aphthae | ** Recurrent genital aphthae | ||
** Eye lesions | ** Eye lesions | ||
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* ESR/CRP elevated | * ESR/CRP elevated | ||
==Management== | ==Management== | ||
* Rheumatology consult +/- multidisciplinary consults | |||
* Oral and genital ulcers: Topical steroid | * Oral and genital ulcers: Topical steroid | ||
* Severe mucocutaneous disease: Prednisone 1mg/kg, low-dose thalidomide, or methotrexate | * Severe mucocutaneous disease: Prednisone 1mg/kg, low-dose thalidomide, or methotrexate | ||
* Systemic disease: Steroid +/- cyclophosphamide or azathioprine | * Systemic disease: Steroid +/- cyclophosphamide or azathioprine | ||
* Ocular: Prednisone + azathioprine and rapid ophthalmologist referral | * Ocular: Prednisone + azathioprine and rapid ophthalmologist referral | ||
* Cerebral venous sinus thrombosis- | * Cerebral venous sinus thrombosis- Heparinization | ||
==Sources== | ==Sources== | ||
<references/> | <references/> | ||
Revision as of 07:20, 23 December 2014
Background
- Chronic small-vessel vasculitis
- Mucocutaneous, ocular, CV, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement
- Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations
Clinical Features
- Age 20-30s MC
- Turkey, Japan, Middle East
- Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis
- Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis
- Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon
- Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia
- GI: Ulcers, obstruction, ileocecal perforation
- Inflammatory oligoarthritis
- Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis
- Cardiac: Myocarditis, endocarditis, pericarditis
- Renal: Glomerulonephritis, amyloidosis
- Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm
Differential Diagnosis
- HSV, lichen planus, pemphigus vulgaris, pemphigoid, IVD, SJS, celiac dz, SLE, auto-inflammatory disorders
- Sjogren's, MS, sarcoidosis, syphillis, TB, malignancy, reactive arthritis, HIV, vasculitides
Classification
- International Study Group Criteria
- Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases:
- Recurrent genital aphthae
- Eye lesions
- Skin lesions
- A positive pathery test
Workup
- Clinical diagnosis
- ESR/CRP elevated
Management
- Rheumatology consult +/- multidisciplinary consults
- Oral and genital ulcers: Topical steroid
- Severe mucocutaneous disease: Prednisone 1mg/kg, low-dose thalidomide, or methotrexate
- Systemic disease: Steroid +/- cyclophosphamide or azathioprine
- Ocular: Prednisone + azathioprine and rapid ophthalmologist referral
- Cerebral venous sinus thrombosis- Heparinization
Sources
- Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p
- Smith EL, et al. Clinical manifestations and diagnosis of Behcet's disease. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
- Alnaimat FA, et al. (2014, Dec 16). Behcet Disease. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/329099-overview