<prompt> 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:Behcets diseasePemphigus vulgarisFixed drug eruptionHerpes labialis  <response>: . Ans. a. Behcets disease Ref: Harrison I9ep2194, ISe p2801Diagnosis 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.Behcets Syndrome Behcets syndrome is chronic multisystem relapsing vascular inflammatory disease vasculitis of unknow n origin Behcets 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 affectedBehcets 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 weeksRECURRENT GENITAL ULCERS: Less common but more specific Do not affect glans or urethra Produce scars scrotal scarsEYE 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 Behcets, 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 Crohns disease 