Ulcus vulvae acutum Ulcus vulvae acutum L. Torok, K Domjan and E. Farag6 SUMMARY A case of a 17 year-old girl with genital ulcers diagnosed as acute vulvar ulcer is reported. No relevant etiologic factors could be established. The gangrenous form of the disease, cutaneous anergy, and the cultured anaerobic pathogens suggest the possibility of infectious pathogenesis. Introduction In 1913 an Austrian dermatologist named Lipschiitz first identified an acute disease with fever, ulceration of the external genital organs, and lymphadenomegaly, which occurs in young women. The syndrome has been termed acute vulvar ulcer. Despite the characteristic symptoms, the disease is ve1y rare and often misdiagno- sed. The following typical case history is a good illustra- tion of this interesting clinical entity. Case history The patient was a 17 year old female. As an infant, she had frequently had respiratory infections with fever and complications. At school, the patient exhibited sym- ptoms of optical atrophy, transient hemiparesis , Ray- naud symptoms, articular complaints, splenomegaly, and lymphadenomegaly. Diagnosis at observation: not further defined autoimmune disease. Complaints at admission: high fever (39°C) of sudden onset and malaise, which persisted for four days. Later, tenderness and swelling of the external genital organs occured, followed by development of very pain- ful nodules and "wounds" impeding walk and urination. The patient never had sexual contacts. She did not complain of any kine.! of respirat01y infection or other interna! disease. Status at admission: slightly swollen and erythe- matous vulva. An ulcer of about 10 mm in diameter on the right labium majus and three ulcers of 15 mm in diameter each, distributed over both labia minora at the vestibulum. The pressure-sensitive, round or elliptical ulcers had sharp edges and were covered with grayish-yellowish crusts. The hymen was intact. The firm inguinal lymph nodes were 1,5-2 cm in diameter. Case report 30 Acta Dermatoven APA Vol 9, 2000, No 1 Case report Laboratory data Erythrocyte sedimentation rate: 47 mm/h; WBC 5000; mild anemia. Urine: ++++ leukocytes. Immune status: Mantoux test and Multitest: complete anergy. Pathergic test negative. Serologic tests: RPR negative, Monostikon test: negative . Epstein Barr virus, Hetpes simplex, Varicella zoster virus: negative. Microscopy of pathogens from the genital ulcers: Neisseria gonorr- hoeae (methylene blue and Gram staining) negative. Bacterial culture: Bacteroides fragilis, Porhyromonas asaccharolyticus and Peptostreptococcus anerobius positive. Trichomonas (native study): negative. Fungi: negative; Mycoplasma, Ureaplasma urealyticum, Chlamydia (ELISA): negative. Herpes virus, EBV: negative. Course ojthe disease and therapy Antibiotic treatment with Doxycyclin 100 mg b.i.d. was introduced. External treatment (sitz bath) with potassium permanganate; Betadine dressings and Betadine ointment. By the second day of treatment the ulcers began to colliquate, and by the third day slou- ghed, leaving behind defects. Pain disappeared after a week, dysuria remaining the most persisting complaint. By the end of the third week the ulcers healed leaving scarred retracted areas. Discussion The clinical entity of hyperacute genital ulceration generally occurs in young women, predominantly virgins (1), although cases of ulceration developing after sexual intercourse (defloration) have also been reported. Similar symptoms have been observed in wives of healthy military staff, who had sexual contacts with their husbands following long periods of absence (2). The cause and pathogenesis of the disease still remains unknown, and only some hypotheses are discu- ssed in the literature. LipschiHz assumed that the disease is caused by autoinoculation with Bacillus crassuss (Doderlein 's lactobacillus), while other physicians of his generation ascribed the disease to poor hygiene of the young women (3, 4). In some cases, Epstein-Barr virusand Ureaplasmawere identified. Recently, genital ulceration ve1y similar to acute vulvar ulcer bas been found in HIV-positive women. These ulcers healed after zidovudin therapy (5, 6, 7). The clinical picture starts with sudclen onset of fever, chills ancl malaise without any prodrome. 3-4 days later, Acta Dermatoven APA Vol 9, 2000, No 1 Ulcus vulvae acutum painful ulcers develop on the interna! surface (vesti- bulum) of the labia minora, but they can affect the labia majora as well. The symptoms are accompanied by dysuria. Basecl on clinical course ancl macromorpho- logic findings, three main forms of the course of the disease can be iclentified: l. Gangrenous form: characteristic hyperacute onset, ulcers covered with grayish-yellowish crusts, extreme pain. Crusts slough after severa! days with scarring. This is is the most frequent form. 2. Chronic form: (pseudo-veneric Lipschiitz ulcer, Scherber's pseuclo-tuberculotic form). A relapsing form with marked edema, circular or partially undercut superficial ulcers. The ulcers usually heal in 4-6 weeks. 3. Miliary form: Purulent, fibrinous ulcers with infla- mmatory edges of pinheacl size. The ulcers typically affect the margins of the labia majora ancl minora, as well as the perineal region. The general symptoms are mild and healing is rapid. Apart from the characteristic labial localization, the involvement of the vagina and the urethra have also been reported. Aphthous ulcers of the buccal mucosa developing together with polymorphic and noclose erythema can be regardecl as an extragenital form of this disease (8) and may possibly be includecl into Behcet's syndrome or a primary herpes infection. Histolopathology is of no diagnostic value (7). The upper part of the edematous corium reveals dilated capillaries with a lymphocyte, histiocyte, plasmocyte ancl fibroblast infiltrate. Proliferation ancl local thicke- ning of the vessel wall is also observecl. In aclvancecl cases, the infiltrate contains mainly polymorphic neutro- phils with subsequent formation of a miliary pseuclo- abscess and ulceration. The disease should be differentiated from venereal (syphilis, ulcus molle) and non-venereal infections (herpes simplex, herpes zoster, miliar ulcerative tuber- culosis). Of the non-infectious diseases, Beh1:et's syn- drome, various aphtous ulcers, ano-vaginal fistula, Mb. Reiter, and myeloproliferative cliseases shoulcl be primarily considered (8). Evaluation of the entire skin and mucosal surface may help in the differential diagnosis. Therapy: Because the etiopathogenesis remains unclear, therapy is mainly symptomatic. Bed rest is important. In acute gangrenous forms, wide-spectrum antibiotic therapy has proved to be beneficial. It should however be emphasizecl that due to the hyperacute character of the disease, its course can scarcely be affec- ted after onset. In severe Beh1:et syndrome-like forms steroids and colchicin therapy should be considerecl. The use of externally applied desinfectants and oint- ments stimulating epithelization is worthwhile. The presented case corresponded to the most fre- quently occurring gangrenous form and may have been 31 Ulcus vulvae acutum Figure 1-2: crusty ulcers affecting the vestibulum and the labia majora Figure 3: tissue lesions left after sloughing of the necrotic tissue induced by a background infection. The latter sugge- stion is supported by the presence of anaerobic patho- gens detected by bacteriological study; these microorga- nisms, however, can not be unambiguously considered as pathogens, since they can occur in normal vagina! flora. We failed to identify any other pathogens (Herpes simplex, Herpes zoster, Epstein-Barr viruses, Myco- plasma or Chlamydia) as mentioned by other authors . Together with the lack of other symptoms, the negative pathergy test excludes Beh~et's syndrome. Cutaneous anergy of the patient may have contributed to the infection by facultative anaerobic pathogens. Acknowledgments The authors express their gratitude for performing the serologic and direct identification studies of the viruses, to the Department of Virology (OKI, Buda- pest) and appreciate the help ofthe Laboratory ofMicro- biology of the Medical Health Service (ANTSZ), Kecskemet. 32 Case report Acta Dermatoven APA Vol 9, 2000, No 1 Cas e r eport Ulcus vulvae acutum R E F E H E N C E S l. Lipschfitz B. iiber eine eigenartige Geschwtirsform des weiblichen Genitales (Ulcus vulvae acutum). AUTHORS' ADDRESSES Acta Dermatoven APA Vol 9, 2000, No 1 Archiv fiir Dermatologie und Syphilis (Berlin) 1913; 114: 363-395. 2. Boyce DC, Valprey JM. Acute ulcerative vulvitis of obscure etiology. Obstet Gynecol 1971; 38: 440-3. 3. Berlin C. The pathogenesis of the so-called ulcus vulvae acutum. Acta Derm Venereol 1965; 45: 221- 2. 4. Gottron HA. , und Schonfeld \Y/. Dermatologie und Venerologie Georg Thieme Verlag- Stuttgart 1965. 5. Brown ZA, Stenchever MA. Genital ulceration and infectious mononucleosis. AM J Obstet Gynecol 1977; 127: 673-4. 6. Portinoy J, Aronheim GA, Chibu F et. al. Recovery of Epstein-Barr virus from genital ulcers. New Engl J Med 1984; 311: 966-8. 7. Covino JM, McCormack WM. Vulvar ulcer of unknown etiology in a human immunodeficiency virus- infected woman, response to treatment with zidovudine. Am J Obstet Gynecol 1990; 163: 115-8. 8. Josey WE.: Anovaginal fistula presenting asa vulvar ulcer. A report of two cases in postmenopausal women. J Reprod Med 1988; 33: 857-8. 9. Daunt S O'N, Kotowski KE, O'Relly AP et al. Ulcerative vulvitis in Reiter's syndrome. Br J Vener Dis 1982; 58: 405-7. Laszl6 Tarok MD, PhD, projessor and chairman, Dpt. Dermatology, County Hospital, Bacs-Kiskum, Nagykorosi 15, H-6000 J{ecskemet, Hungary Kornelia Domjan MD, same address Eszter Farag6 MD, same address 33