Behcet's disease: Difference between revisions
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==Background== | ==Background== | ||
[[File:Behcet's.png|thumb|]] | [[File:Behcet's.png|thumb|]] | ||
*Chronic small-vessel vasculitis | *Chronic small-vessel [[vasculitis]] | ||
*Mucocutaneous, ocular, cardiovascular, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement | *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 | *Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations | ||
==Clinical Features== | ==Clinical Features== | ||
*Age 20-30s | *Age 20-30s most commonly | ||
*Turkey, Japan, Middle East | *More common in Turkey, Japan, Middle East | ||
*Triad: Recurrent oral aphthous ulcers, genital ulcers, and [[uveitis]] | *Triad: Recurrent oral aphthous ulcers, genital ulcers, and [[uveitis]] | ||
*Classically painful ulcer with necrotic center and red rim | *Classically painful ulcer with necrotic center and red rim | ||
*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 | ||
*Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, [[cerebral sinus thrombosis]], optic nerve ischemia | *Neurologic: Brainstem and corticospinal tract syndromes, aseptic [[meningoencephalitis]], [[increased ICP]], [[cerebral sinus thrombosis]], optic nerve ischemia | ||
*GI: Ulcers, obstruction, ileocecal perforation | *GI: Ulcers, obstruction, ileocecal perforation | ||
*Inflammatory | *Inflammatory oligo[[arthritis]] | ||
*Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis | *Vasculopathy: Aneurysm, [[vasculitis]], superficial [[thrombophlebitis]], thrombosis | ||
*Cardiac: Myocarditis, endocarditis, pericarditis | *Cardiac: [[Myocarditis]], [[endocarditis]], [[pericarditis]] | ||
*Renal: Glomerulonephritis, amyloidosis | *Renal: [[Glomerulonephritis]], amyloidosis | ||
*Pulmonary: Pleural effusions, pulmonary hypertension, pulmonary aneurysm | *Pulmonary: [[Pleural effusions]], [[pulmonary hypertension]], pulmonary aneurysm | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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*celiac disease | *celiac disease | ||
*[[SLE]] | *[[SLE]] | ||
*[[Sjogren's]] | *[[Sjogren's]] | ||
*[[MS]] | *[[MS]] | ||
*[[ | *[[Sarcoidosis]] | ||
*[[ | *[[Syphilis]] | ||
*[[TB]] | *[[TB]] | ||
*malignancy | *malignancy | ||
* | *[[Reactive arthritis]] | ||
*[[HIV]] | *[[HIV]] | ||
* | *Other [[vasculitis syndromes]] | ||
==Evaluation== | ==Evaluation== | ||
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==Management== | ==Management== | ||
*Rheumatology consult +/- multidisciplinary consults | *Rheumatology consult +/- multidisciplinary consults | ||
*Oral and genital ulcers: Topical | *Oral and genital ulcers: [[Topical steroids]] | ||
*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]]- [[heparin]] | ||
==References== | ==References== | ||
Revision as of 23:12, 13 November 2016
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
- pemphigoid
- IVD
- SJS
- celiac disease
- SLE
- Sjogren's
- 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