Behçet or not Behçet ? Dermatology quiz Camille Frances Hôpital Tenon

Behçet or not Behçet ?
Dermatology quiz
Camille Frances
Hôpital Tenon
[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,
 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
What is the diagnosis ?
Acute Genital Ulcers of Lipschütz
 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
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)
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
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
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
Oral ulceration
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
 Biopsy of jugal ulceration: non specific
 Positive ANCA (Proteinase 3 type)
Oral ulcers in granulomatosis with polyangiitis
Frequent : 10% to 50% of cases
Unlike aphthae, persistent and not
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