A man presents with a 6-month history of recurrent oral ulceration. He has yellowish ulcerations on his lip which are erythematous, with a central halo and nodular lesions on his shin. Diagnosis is:
. Ans. a. Behcet's disease (Ref: Harrison I9/ep2194, IS/e p2801)Diagnosis of a patient who presents with a 6-month history of recurrent oral ulceration, yellowish ulcerations on the lip, erythematous, with a central halo and nodular lesions on shin is Behcetdisease.Behcet's Syndrome* Behcet's syndrome is chronic multisystem relapsing vascular inflammatory disease (vasculitis) of unknow n origin* Behcet's disease is primarily a small vessel vasculitis but it can also involve large vessels both arteries and veins.* Associated with HLA-B5* Disease affect young patients: Both males and females are equally affectedBehcet's SyndromeDiagnostic CriteriaEssential criteria + Two non-essentia! criteria are required for diagnosisEssential criteriaNon-essentia! criteriaRECURRENT ORAL ULCERATIONS:* Oral ulceration is the hallmark of the disease* Sin qua non for diagnosis* Types of ulcers: Minor aphthous, major aphthous and herpetiform ulcers* Features of Oral ulcers:- PainfulShallow with a central yellowish necrotic base- Appear singly or in crops- Subside without leaving scars (persist for 1-2 weeks)RECURRENT GENITAL ULCERS:* Less common but more specific* Do not affect glans or urethra* Produce scars (scrotal scars)EYE LESIONS:* Anterior uveitis (recurrent hypopyon)* Posterior uveitis, Retinal vasculitisSKIN LESIONS:* Erythema nodosum, Pseudofollicullitis* Pseudopustular lesions, Acneiform nodules POSITIVE PATH ERG Y TEST:* Pathergy refers to hyperactivity of skin to any intracutaneous injection or needle stick* Pathergy is characteristic feature of Behcet's, although the mechanism is unknown* Pathergy is said to be positive if a papule or pustule >2 mm develops 24-48 hours after needle prick to a depth of 5 mm. Other Clinical Features* Arthritis- Non-deforming arthritis commonly affects knees and ankles* Vascular Lesions (Thrombophlebitis):- Superficial or deep peripheral vein thrombosis (25%)- Arterial thrombosis may be seen* Neurological Involvement (5-10%)- Dural vein thrombosis* Pulmonary symptoms:- Pulmonary artery vasculitis may occur and present with dyspnea, cough, chest pain, hemoptysis* Gastrointestinal involvement:- Mucosal ulceration of gut similar to Crohn's disease
Behcet's disease
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