Behcet's disease: Difference between revisions
Amyamamoto (talk | contribs) (Created page with "==Background== ==Clinical Features== ==Differential Diagnosis== ==Workup== ==Management== ==Disposition== ==See Also== ==External Links== ==Sources== <references/>") |
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==Background== | ==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== | ==Clinical Features== | ||
* Age 20-30s MC | |||
* Turkey, Japan, Middle East | |||
* Triad: 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 | |||
==Differential Diagnosis== | ==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 systemi diseases: | |||
** Recurrent genital aphthae | |||
** Eye lesions | |||
** Skin lesions | |||
** A positive pathery test | |||
==Workup== | ==Workup== | ||
* Clinical diagnosis | |||
* ESR/CRP elevated | |||
==Management== | ==Management== | ||
* 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- Heparin | |||
==Disposition== | ==Disposition== | ||
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==Sources== | ==Sources== | ||
<references/> | <references/> | ||
*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 | |||
Revision as of 07:00, 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: 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
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 systemi diseases:
- Recurrent genital aphthae
- Eye lesions
- Skin lesions
- A positive pathery test
Workup
- Clinical diagnosis
- ESR/CRP elevated
Management
- 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- Heparin
Disposition
See Also
External Links
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