Behcet's disease: Difference between revisions
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* Renal: Glomerulonephritis, amyloidosis | * Renal: Glomerulonephritis, amyloidosis | ||
* Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm | * 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 | |||
==Diagnosis== | |||
* Clinical diagnosis | |||
* ESR/CRP elevated | |||
===Classification=== | ===Classification=== | ||
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** Skin lesions | ** Skin lesions | ||
** A positive pathery test | ** A positive pathery test | ||
==Management== | ==Management== | ||
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* Cerebral [[venous sinus thrombosis]]- Heparinization | * Cerebral [[venous sinus thrombosis]]- Heparinization | ||
== | ==References== | ||
<references/> | <references/> | ||
*Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540. | *Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540. | ||
Revision as of 13:46, 20 July 2015
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
Diagnosis
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
Diagnosis
- Clinical diagnosis
- ESR/CRP elevated
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
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
References
- Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540.
- 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