Behçet's disease: Difference between revisions
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[HSV]] | *[[HSV]] | ||
*[[ | *[[Lichen planus]] | ||
*[[ | *[[Pemphigus vulgaris]] | ||
*[[pemphigoid]] | *[[Bullous pemphigoid]] | ||
*IVD | *IVD | ||
*[[SJS]] | *[[SJS]] | ||
*celiac disease | *celiac disease | ||
*[[SLE]] | *[[SLE]] | ||
*[[ | *[[Sjögren Syndrome]] | ||
*[[MS]] | *[[MS]] | ||
*[[Sarcoidosis]] | *[[Sarcoidosis]] | ||
*[[Syphilis]] | *[[Syphilis]] | ||
*[[TB]] | *[[TB]] | ||
* | *Malignancy | ||
*[[Reactive arthritis]] | *[[Reactive arthritis]] | ||
*[[HIV]] | *[[HIV]] | ||
*Other [[vasculitis | *Other [[vasculitis]] syndromes | ||
==Evaluation== | ==Evaluation== | ||
Line 56: | Line 56: | ||
*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]]- [[heparin]] | *Cerebral [[venous sinus thrombosis]]- [[heparin]] | ||
Revision as of 23:32, 17 October 2019
Background
- Chronic small-vessel vasculitis
- Mucocutaneous, ocular, cardiovascular, 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 most commonly
- More common in Turkey, Japan, Middle East
- Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis
- Classically painful ulcer with necrotic center and red rim
- 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 hypertension, pulmonary aneurysm
Differential Diagnosis
- HSV
- Lichen planus
- Pemphigus vulgaris
- Bullous pemphigoid
- IVD
- SJS
- celiac disease
- SLE
- Sjögren Syndrome
- MS
- Sarcoidosis
- Syphilis
- TB
- Malignancy
- Reactive arthritis
- HIV
- Other vasculitis syndromes
Evaluation
- 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 steroids
- 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- heparin
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