Acta Dermato-Venereologica issue 50:1 98-1CompleteContent | Page 35

134 SHORT COMMUNICATION Epstein-Barr Virus-related Acute Genital Ulcer Successfully Treated with Colchicine Agathe NOUCHI 1 , Gentiane MONSEL 1 , Barthélémy LAFON-DESMURS 1 , Laurence MENG 2 , Sonia BURREL 3 , Micheline MOYAL- BARRACCO 4 and Eric CAUMES 1 Departments of 1 Infectious and Tropical Diseases, 2 Gynaecology and 3 Virology, Hôpital Pitié-Salpétrière, 45-83 Bd de l’hôpital, FR-75013 Paris, and 4 Department of Dermatology, Hôpital Cochin, Paris, France. E-mail: [email protected] Accepted Aug 9, 2017; Epub ahead of print Aug 10, 2017 Acute genital ulcer (AGU), also known as reactive non-sexually-related acute genital ulcers, ulcus vulvae acutum or Lipschütz’s ulcer, was first described in 1913. This non-venereal acute genital ulceration, of unknown aetiology, is associated with infections caused by various microorganism s, in particular acute Epstein-Barr virus (EBV) infection (1–4). The ulceration usually heals spontaneously within 2–4 weeks, but treatment may be necessary in severe cases. We report here a case of AGU due to reactivation of EBV, which was treated successfully with colchicine, whereas other treatments failed. CASE REPORT A previously healthy, Caucasian, non-virgin, 18-year-old female presented with a 2-week history of fever, malaise, nausea and sore throat, and a 10-day history of intense vulvar pain, followed by acute vulvar ulcerations. Treatment with valacyclovir, started by her general practitioner, had no effect on the vulvar lesions. She was then referred to our department and hospitalized for pain management. Any traumatic precipitating factor was denied. She did not report any personal or familial history of oral or ge- nital aphthous or digestive disorders. On physical examination, she had enlarged and erythematous tonsils. Her oral mucosa was normal. Wide, isolated or confluent, well-circumscribed ulcers were present on the fourchette and on the inner posterior aspects of the labia majora, in a bilateral kissing pattern (Fig 1a). The periphery of the ulcers was red, whereas their surface was yel- low due to fibrin deposit. There was no further fever and no other systemic signs of infection. Laboratory findings showed a mononucleosis syndrome with 10,850/mm 3 white blood cells including 5,420/mm 3 lymphocytes (50%) and 1,080/mm 3 monocytes (10%) and presence of hyper- Fig. 1. Clinical findings. (a) Wide, well-circumscribed ulcers of the fourchette and inner posterior aspects of the labia majora. (b) Almost complete ulcer healing on the 7 th day of treatment. doi: 10.2340/00015555-2761 Acta Derm Venereol 2018; 98: 134–135 basophilic atypical lymphocytes. Serological tests for Treponema pallidum, human immunodeficiency virus, cytomegalovirus (CMV), herpes simplex virus (HSV) and toxoplasmosis were negative. Bacteriological analysis of genital swabs was negative, as well as PCR on genital lesions for HSV 1 and 2, varicella-zoster virus, CMV and EBV. EBV serology was positive for IgM against viral capside antigen (VCA) and IgG against VCA and the nuclear antigen (EBNA), in favour of viral reactivation. PCR for EBV on blood was weakly positive at 2.9log (801 copies/ml). We decided not to perform a vulvar biopsy due to the limited diagnostic value and the invasiveness of this procedure. Opioids and topical lidocaine could not reduce the patient’s intense pain, causing dysuria and requiring a urinary catheter. Topical and systemic corticosteroids (0.5 mg/kg/day for 3 days) brought no improvement. Topical imiquimod for 3 days was also not effective and, 20 days after onset, new ulcerations occurred on the inner aspect of both labia minora. Treatment with colchine, 1 mg per day, was therefore introduced, leading to a rapid reduc- tion in pain intensity and almost complete ulcer healing on the 7 th day of treatment (Fig. 1b). Treatment was stopped after 10 days, with no relapse during the 24-months follow-up. A control of EBV serology performed one month later revealed the disap- pearance of IgM anti-VCA with persistence of IgG anti-VCA and anti-EBNA, whereas the EBV PCR on blood was negative, all of which confirmed reactivation of EBV. DISCUSSION Although the aetiologies and pathogenic mechanism of AGU are not fully understood, and some authors still do not distinguish AGU from vulvar aphthosis (5, 6), the term AGU should be used to refer only to non-venereal genital ulceration that appears to occur suddenly in response to a systemic infection, with exclusion of idiopathic and complex aphthosis and chronic systemic diseases, such as Behçet’s and Crohn’s diseases (1). The association of some cases of AGU with EBV infec- tion was suggested in 1977 and, since then, approximately 40 EBV-linked cases have been reported, mostly during primary infection or unspecified EBV infection (4), the involvement of EBV being reported in approximately 30% of AGU (1). Other infectious agents have also been involved in some cases, including CMV (7), Toxoplasma gondii, Mycoplasma pneumonia (6), Streptococcus spp, Salmonella enterica (8), mumps, influenza A and B virus, adenovirus (9), and Borrelia burgdorferi (10). AGU typically presents with acute, very painful, well-circumscribed fibrinous or necrotic vulvar ulcers in a “kissing pattern” (1–4), usually preceded by flu- like systemic signs, such as fever, headache, fatigue or tonsillitis, occurring in immunocompetent young girls. However, the mean age of 15.1 years reported in the This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.