Behçet or not Behçet ? Dermatology quiz Camille Frances Hôpital Tenon France [email protected] Case 1  Previously healthy 19 year old woman  Acute painful genital ulcers with urinary retention due to pain  Odynophagia for 10 days, intense fatigue  No previous sexual contact  At physical examination  fever 40°C  pharyngeal oedema  lymphadenopathies Laboratory investigations  Blood count: increased lymphocytes 6500/ml  Negative bacterial cultures from blood, ulcerations, pharynx  Negative viral culture for HSV from vulvar ulcerations  Negative serologic tests for CMV, HIV, viral hepatitis B and C, syphilis, toxoplasmosis, p24 antigenemia  Transaminases x2N  Vulvar biopsy: non specific inflammatory dermal infiltrate What is the diagnosis ? Diagnosis Acute Genital Ulcers of Lipschütz Follow  At day 10, positive anti-EBV capsid antibodies (IgM)  At day 18, positive anti-EBV capsid antibodies (IgM and IgG)  Negative anti-EBV nuclear antibodies (IgM or IgG)  EBV serum load : 425 copies/ml  Acute Genital Ulcers of Lipschütz with EBV primary infection Acute Genital Ulcers of Lipschütz Fahri D et al. Arch Dermatol 2009; 145: 38-45      Acute genital ulcers with the absence of further relapses No past history of recurrent aphthae Frequently young girls <20 year old without sexual activity Bilateral vulvar ulcers with a kissing pattern Spontaneous healing (but frequently antibiotic and antiviral therapy)  Duration of lesions < 30 days  Fever, myalgias  Mild liver abnormalities Associated infections           EBV primary infection (1/3) Salmonellosis Mycoplasmas HIV, Mumps Cytomegalovirus Influenza A Toxoplasma gondii Lyme disease Finch JJ et al Jama Dermatol 2014 on line Not precised in many cases Case 2  Previously non smoker, healthy, French, 21 yearold man  Without past medical history  Painful lesions on the lower right side vestibule for 1 month and on penis for 15 days  No general manifestation, no skin lesion  Absence of ocular or gastrointestinal complaint What to do during clinical examination for diagnosis ? Aphthae in chronic inflammatory bowel disease     Incidence : 5 to 30% Chronic ulcerative colitis > Crohn’s disease Unipolar, bipolar or tripolar (mouth, genitalia, anus) In less than 10% of cases, parallel evolution with gastrointestinal involvement  May be associated with specific granulomatous lesions of Crohn’s disease  May precede by several years the gastrointestinal involvement Case 3  French 55 year old man  Past history:    chronic sinusitis from 3 years; Atherosclerosis with transient ischemic attacks 2 years before (heavy smoker) Treatment: aspirin 100mg/d, nicorandil 30mg/d, rosuvastatine 10mg/d for 2 years + antibiotics and steroids during flares of sinusitis  Jugal ulceration from 1 month  Fever, purpuric lesions on the legs What possible diagnoses ?     Behcet’s disease Crohn’s disease Granulomatosis with polyangiitis (Wegener) Nicorandil-induced mucosal ulceration and purpura What possible diagnoses ?  Behcet’s disease : Possible but unlikely   Too old for Behcet’s disease onset Purpura: rarely an initial manifestation  Crohn’s disease : Possible but unlikely    Granulomatosis with polyangiitis : Yes      Normal anus No gastrointestinal complaint Sinusitis, Oral ulceration Purpura Fever Nicorandil-induced dermatologic lesions: Unlikely   Jugal ulceration may be induced by nicorandil Onset of purpuric lesions is too late as nicorandil is taken for 2 years Granulomatosis with polyangiitis  Biopsy of purpuric lesions : leukocytoclastic vasculitis  Biopsy of jugal ulceration: non specific inflammation  Positive ANCA (Proteinase 3 type) Oral ulcers in granulomatosis with polyangiitis     Frequent : 10% to 50% of cases Unlike aphthae, persistent and not recurrent Number and location highly variable (cheeks, tongue, floor of the mouth, lips, palate, gingivae, tonsils, posterior palate) Pathologic findings usually nonspecific : acute and chronic inflammation, rarely: extravascular granuloma Thank you