Ulcus vulvae acutum - A rare case - UI Scholars Hub

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Journal of General - Procedural Dermatology & Venereology Journal of General - Procedural Dermatology & Venereology Indonesia

Indonesia

Volume 3

Number 2 June Edition Article 6

6-30-2019

Ulcus vulvae acutum - A rare case Ulcus vulvae acutum - A rare case

Irene Dorthy Santoso

Department of Dermatology and Venereology, Faculty of Medicine Universitas Indonesia Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia

Ika Anggraini

Wresti Indriatmi

Correspondence: Wresti Indriatmi; Department of Dermatology and Venereology, Faculty of Medicine Universitas Indonesia, Dr. Cipto Mangunkusumo National General Hospital, Jakarta, Indonesia Yudo Irawan

Hanny Nilasari

See next page for additional authors

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Part of the Dermatology Commons, Integumentary System Commons, and the Skin and Connective Tissue Diseases Commons

Recommended Citation Recommended Citation

Santoso, Irene Dorthy; Anggraini, Ika; Indriatmi, Wresti; Irawan, Yudo; Nilasari, Hanny; Marissa, Melani;

Sirait, Sondang P.; and Rihatmadja, Rahadi (2019) "Ulcus vulvae acutum - A rare case," Journal of General - Procedural Dermatology & Venereology Indonesia: Vol. 3: No. 2, Article 6.

DOI: 10.19100/jdvi.v3i2.165

Available at: https://scholarhub.ui.ac.id/jdvi/vol3/iss2/6

This Article is brought to you for free and open access by UI Scholars Hub. It has been accepted for inclusion in Journal of General - Procedural Dermatology & Venereology Indonesia by an authorized editor of UI Scholars Hub.

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Ulcus vulvae acutum - A rare case Ulcus vulvae acutum - A rare case

Authors Authors

Irene Dorthy Santoso, Ika Anggraini, Wresti Indriatmi, Yudo Irawan, Hanny Nilasari, Melani Marissa, Sondang P. Sirait, and Rahadi Rihatmadja

This article is available in Journal of General - Procedural Dermatology & Venereology Indonesia:

https://scholarhub.ui.ac.id/jdvi/vol3/iss2/6

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J Gen Proced Dermatol Venereol Indones. 2019:3(2);30-34. 30 Case Report

Ulcus vulvae acutum – A rare case

Irene Dorthy Santoso, Ika Anggraini, Wresti Indriatmi*, Yudo Irawan, Hanny Nilasari, Melani Marissa, Sondang P. Sirait, Rahadi Rihatmadja

*Correspondence: Wresti Indriatmi.

Email: [email protected]

Abstract

Background: Ulcus vulvae acutum, also known as Lipschütz ulcer, is a rare condition characterized by multiple, painful, and acute genital ulceration in young women without venereal infections, associated with systemic symptoms like malaise, fever, and inguinal lymphadenopathy.

Case Illustration: A 24-year-old woman developed multiple and painful vulvar ulcers that recurred for almost a year. The patient was primarily diagnosed and treated as genital herpes in several hospitals with only slight improvement and no complete healing. The patient came to our hospital for examinations to rule out sexually- transmitted infection (STI) (genital herpes, syphilis, HIV, and chancroid) and possibility of Behçet’s. The patient was treated with a combination of 2.5% lidocaine and 2.5% prilocaine creams for 1 week, 2% fusidic acid ointment for 1 week, with no significant progression. Later, the therapy was changed to 0.05% clobetasol propionate ointment for 10 days. Complete healing occurred in about 10 weeks with no scarring.

Discussion: Diagnosis of ulcus vulvae acutum in this patient was made by excluding other differential diagnoses.

Conclusion: Ulcus vulvae acutum is a difficult diagnosis, and clinician should be aware of this entity especially when encountering young woman without previous sexual experience problem with ulcer.

Keywords: ulcus vulvae acutum, genital ulcer, young women

Background

In 1913, an Austrian dermatologist Benjamin Lipschütz reported a case of young girl who developed vulvar ulcers without a clear infection source. Lipschütz ulcers (LU) refers to an ulceration of the vulva or lower vagina without venereal origin, also known as acute genital ulcer or ulcus vulva acutum.^1,2 Ulcus vulvae acutum is a rare condition characterized by painful, multiple, acute genital ulceration in young women without venereal infection, associated with systemic symptoms like malaise, fever, and inguinal lymphadenopathy.^3,4 Usually ulcus vulvae acutum occurred in young girls between 14-20 years.⁵ The etiology and pathogenesis of ulcus vulvae acutum remains unknown. Differential diagnosis of ulcus vulvae acutum includes sexually- and non- sexually-transmitted infections. The goals of treatment for ulcus vulvae acutum are to improve healing, prevent scarring, and reduce pain.

Case Illustration

A 24-year-old woman was consulted to STI-clinic with a diagnosis of recurrent genital herpes infection. The patient’s chief complaint was painful ulcers on the labia majora for almost 1.5 years, especially close to her menstrual period. The patient was already treated by other dermatologist with oral antiviral agents and topical creams, with only slight improvement and no complete healing.

Every time the ulcer appeared, the patient was given 200 mg acyclovir for five times a day, but the ulcer never resolved. About two months ago, the patient came to our Department of Dermatology and Venereology in Dr. Cipto Mangunkusumo National General Hospital, Jakarta with the same complaint.

We asked the patient about her previous illness and the patient had never had an oral sores, blurred vision, or bowel disorder. The patient and her husband had never have sexually-transmitted

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J Gen Proced Dermatol Venereol Indones. 2019:3(2);30-34. 31 diseases before. Patient had been married for 1.5

years, and wanted to have child immediately. This couple had never had pre-marital sexual experience. Their sexual intercourses were usually genitogenital, sometimes orogenital (fellatio). The patient usually changed her underwear twice daily, used pantyliners and female genital soap every day. The patient is considered healthy with no skin disease. Her menstruation cycle had been regular.

Clinical examination revealed multiple ulcers on the labia majora and minora, lenticular in size, erythematous, not undermined, no ragged edge, sharply demarcated, and covered with pus (Figure 1). No lymphadenopathy was noted. On speculum examination, we found hyperemic cervix with small amount of odorless mucoid discharge. Whiff test was negative, and the pH was 5. From the swab with Gram staining, we found more than 30 white blood cells in the cervix and 10-15 white blood cells in the labia majora. We found no rod-shaped Gram- positive bacteria, and a lot of Gram-positive cocci.

Based on the result of clinical and swab examination, we suspected that the patient has recurrent genital herpes with Behçet’s disease as differential diagnosis and non-specific cervicitis.

The patient was treated with 200 mg acyclovir for five times daily for five days, 2% fusidic acid ointment twice daily for recurrent genital herpes with secondary infection, and for her non-specific cervicitis we did syndromic approach and gave a single dose 1 g azithromycin and single dose 400 mg cefixime. We asked the patient to undergo serology tests for IgM, IgG HSV 1 and HSV 2. We advised abstinence until the lesion healed.

The patient came after a week of treatment and no new lesion was observed. Older lesions were not completely healed, but the volume of discharge was reduced. Clinical examination revealed multiple lenticular erosion and excoriation and no lymphadenopathy was noted. Serologic test for syphilis (TPHA and VDRL) and HIV screening test result of the patient and her husband were non- reactive. Anti HSV-1 and HSV-2 IgG and IgM were also non-reactive. Only the husband’s anti-HSV-1

IgG was reactive. The patient was diagnosed as non-specific cervicitis based on clinical presentation and suspected of Behçet’s disease. A biopsy was performed and later the patient was consulted to the Ophthalmology Department.

Histopathology examination revealed lymphocytic infiltrate around swollen blood vessels. However, this finding was not specific, as they might be seen in other conditions, including ulcus vulvae acutum.

Examination performed by the consulted Ophthalmology Department showed no abnormal findings on both eyes. Therefore, we excluded Behcet’s disease and considered it as recurrent vulvar aphthae. PCR HSV was suggested and the patient was treated with a combination of 2.5%

lidocaine and 2.5% prilocaine cream twice daily, and we proposed PCR HSV examination.

The patient came back after a week of treatment with 2.5% lidocaine and 2.5% prilocaine cream combination, and there were many new painful lesions. Clinical examination of the labia majora and minora showed multiple painful ulcers, lenticular in size, erythematous, not undermined, no ragged edge, sharply demarcated, and covered with pus. No lymphadenopathy was noted. The patient diagnosed as ulcus vulvae acutum with secondary infections. The patient was then treated with 2% fusidic acid ointment twice daily for a week, but there was no significant response to the treatment. The 2% fusidic acid ointment was stopped and switched to 0.05% clobetasol propionate ointment twice daily for 10 days and the patient was given sitz bath with 1:10.000 potassium permanganate solutions twice daily for about twenty minutes.

After ten days of treatment with 0.05% clobetasol propionate ointment, the patient came back and the ulcers had partially improved, though not completely healed. Clinical examination revealed multiple minimal erosion, lenticular in size, not sharply demarcated and no lymphadenopathy was noted (Figure 2). The patient was satisfied with the treatment.

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J Gen Proced Dermatol Venereol Indones. 2019:3(2);30-34. 32

Figure 1. First visit (multiple ulcers)

Figure 2. Ten days after treatment with 0.05% clobetasol propionate ointment

Discussion

The etiology and pathogenesis of ulcus vulvae acutum remain unknown. Lipschütz initially assumed that ulcus vulvae acutum was caused by autoinoculation with Bacillus crassuss (Dőderlein’s lactobacillus), while others considered poor hygiene as a risk factor.^1,6 Previous studies revealed that ulcus vulvae acutum is associated with infectious agents including EBV, CMV, mumps, Mycoplasma pneumonia, salmonellosis, and influenza A, but in most cases, connection with infection could not be confirmed.⁷ Ulcus vulvae acutum can also be triggered by drugs, albeit very rarely. EBV can cause ulcus vulvae acutum through two mechanisms: first, from hematogenous spread or autoinoculation, and second, from type III hypersensitivity reaction to viral or bacterial infections, leading to deposition of immune complexes in the dermal vessels, that can activate the complement system, resulting in microthrombi formation and tissue necrosis.⁸ The clinical presentation of ulcus vulvae acutum consists of a sudden onset of painful vulvar ulcers, single or multiple, usually wider than 1 cm and deep, shallow ulcers with raised, sharply demarcated borders. The ulcers have grey exudate (pseudo-membrane) or adherent grey-black eschar.² Bilateral kissing lesions are characteristic for ulcus vulvae acutum.⁹ This is similar to our patient’s clinical presentation, although without any kissing lesions. In some patients, the ulcers initially present as a red or black pseudovesicles, which then develop as an eschar with underlying painful ulcer. A small area of granulomatous tissue can be seen before the lesion is completely healed.² Ulcus vulvae acutum is usually located at labia minora or

majora (mainly on the inner aspect of the labia minora), perineum, fourchette, and vestibule.^1,10 In addition to its labial localization, the ulcers can also be found in the urethra and vaginal introitus.⁶ Patients usually have systemic symptoms such as fever, headache, myalgia, skin rash, tonsillitis, lymphadenopathy, dysuria, and diarrhea.^1,2 Additionally, clinicians also need to ask about the onset, duration of ulcers, patient’s sexual history, and other diseases. In our patient, we found no systemic symptoms.

Differential diagnoses of ulcus vulvae acutum includes sexually- and non-sexually-transmitted infections, as well as autoimmune, systemic illness, drug reactions, and also idiopathic aphthosis.

Sexually-transmitted infections include HSV, syphilis, chancroid, and HIV. Non-sexually- transmitted infections include herpes simplex virus, Epstein-Barr virus, Cytomegalovirus, Influenza A, and paratyphoid infections. Systemic illness is another differential diagnosis for ulcus vulvae acutum, which may include Crohn’s disease, iron, folate, and vitamin B12 deficiency, and Behçet’s disease. Drug reactions to NSAID can mimic ulcus vulvae acutum. Autoimmune progesterone dermatitis must be excluded to diagnose ulcus vulvae acutum.^2,6,7,10 Referring to these references, we performed blood examination and skin biopsy to exclude the differential diagnosis, and consulted other divisions.

Herpes simplex virus is the most common sexually- transmitted infections. About 10-60% patients with ulcus vulvae acutum had HSV infection. The vulvar manifestations of Crohn’s disease are characterized by knife cut ulcers or fissures, located mainly on the inguinal, gluteal, and

1 2

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J Gen Proced Dermatol Venereol Indones. 2019:3(2);30-34. 33 interlabial folds.² Behçet’s disease is a multisystem

inflammatory disease of unknown etiology, characterized clinically by recurrent oral aphthous, genital ulcer, and skin lesions. There are two main diagnosis criteria for Behçet’s disease: The International Study Group criteria and the criteria developed by the Behçet’s Disease Research Committee of Japan.¹¹ The ulcers of Behçet’s disease are deep and painful, and usually heal with scarring. It may take 6-8 years from the first ulcer to establish the diagnosis of Behçet’s disease.

Ulcus vulvae acutum can imitate autoimmune progesterone dermatitis, which are painful necrotic ulcers that recur in the late luteal phase of menstrual cycle when progesterone levels are high.²

The diagnosis of ulcus vulvae acutum required complete examination, including sexual history and history of systemic illness especially ocular, neurologic, gastrointestinal, and genitourinary symptoms. Clinicians should look for oral and skin lesions, especially in the oropharynx and perianal area. Speculum examination is not routinely done due to the patient’s discomfort, and general anesthesia is occasionally required for evaluation.² No speculum examination was done in this particular case.

Govindan¹ in 2016 presented a literature review on 79 cases of Lipschütz ulcers from 20 studies between 2003 until 2015 and revealed that serology tests for HIV, HSV, hepatitis B and hepatitis C were negative for all of the patients.

Specific serology test to identify EBV, CMV, mumps, mycoplasma, influenza, parvovirus, B19 (PVB19), and paratyphoid were also done. From 79 patients, EBV was positive in 13 patients (16%), CMV in 4 (5%), M. pneumoniae in 4 (5%), M.

fermentans in 3 (3.7%), mumps in 1 (1.2%), PVB19 in 1 (1.2%), paratyphoid in 1 (1.2%), and etiology could not be confirmed for 50 patients (63%).

Similarly, Baptista et al.¹² in 2015 reported a retrospective analysis conducted in a five-year period, from March 2009 until March 2014, and revealed that the minimum study protocol included serology tests for HIV, HSV1 and 2, parvovirus B19 (PVB19), cytomegalovirus (CMV), Epstein-Barr virus (EBV), Toxoplasma gondii and Mycoplasma pneumoniae. A treponemal (TPHA) and/or non- treponemal (VDRL) test was mandatory, and PCR HSV. C-reactive protein, blood counts, and liver enzymes should be performed if there were severe systemic symptoms or delay in wound healing, as they are not specific. Complete blood count may detect anemia, neutropenia, or thrombocytopenia, which can lead to the diagnosis of an underlying disorder.² Blood examination in our patient were as

follows: EBV VCA Ab IgM <36 U/ml (negative), and IgG 182 U/ml (positive; negative if IgG <18 U/ml).

This blood examination supported the diagnosis of ulcus vulvae acutum in this patient. Histopathology examination is not recommended as a first line examination, due to the fact that the result mostly reveals non-specific mixed inflammatory infiltrate in the dermis, as seen in our case. It is more useful to rule out others diseases, if other examinations yield negative result.¹ Microbiologic testing is not routinely recommended as the result may be non- specific. Histopathology and microbiologic examinations are recommended for more than one episode of ulcus vulvae acutum.²

The treatment goals for ulcus vulvae acutum are to improve healing, prevent scarring, and reduce pain. If the underlying disease is established, the treatment should be focused on specific etiology.² Because the etiopathogenesis of this disease remains unclear and specific etiology is difficult to find, the treatment is mainly symptomatic.^6,10 Topical treatment include disinfectants, topical antibiotics, topical anesthetics, and topical corticosteroid. Application of 0.05% clobetasol propionate ointment 2 times daily for 7-10 days, or intralesional injections of 10 mg/mL triamcinolone directly into the ulcer base were recommended depending on the patient’s tolerance.^2,13 Systemic therapy includes oral analgesics (NSAID) with or without broad spectrum antibiotic therapy. In severe and painful forms, oral corticosteroid may be needed. Oral corticosteroid can be prednisone, dexamethasone, or methyl prednisolone with an average dose of 0.5/kg of for 1-2 weeks with tapering off dosage.^1,4,13 Systemic corticosteroid is used when the patient fails to respond to topical agents. Other studies also reported significant improvement with antibiotic therapy with azithromycin and metronidazole. Sitz bath may improve pain score and remove adherent necrotic material.²

The diagnosis of ulcus vulvae acutum in this patient was made by excluding others differential diagnoses. The patient was treated with 0.05%

clobetasol propionate ointment for ten days with satisfactory improvement. Based on the examination result, none of Behçet’s disease criteria were fulfilled. Annual follow up is still needed for this patient to anticipate the possibility of Behçet’s disease. Patients with ulcus vulvae acutum were usually followed up every week until re-epithelialization occurred and the pain has resolved. The ulcer usually resolved between 5-52 days.²

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J Gen Proced Dermatol Venereol Indones. 2019:3(2);30-34. 34

Conclusion

Ulcus vulvae acutum is a rare condition and is difficult to diagnose. Clinicians should be aware of this entity, especially when encountering young women with ulcer and no prior history of STI. As the diagnosis is based on exclusion of other diseases, tests such as STS and herpes virus serology test should be performed.

References

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2016;2(1):73-8.

2. Huppert JS. Lipschütz ulcers: Evaluation and management of acute genital ulcers in women.

Dermatol Ther. 2010;23:533-40.

3. Visentin D, Driul L, Buligan C, et al. Ulcus vulvae acutum – A case of genital ulcers in adolescent girl. Case Reports in Women’s Health.

2016;9:4-6.

4. Trčko K, Belič M, Miljkovič. Ulcus vulvae acutum. Acta Dermatoven APA.

2007;16(4):174-6.

5. Bhat RM, Furtado S. Lipschütz ulcer. Indian J Sex Transm Dis. 2007;28(2):106-7.

6. Tőrők L, Domjăn K, Faragŏ E. Ulcus vulvae acutum. Acta Dermatoven APA. 2000;9(1):30- 3.

7. Kinyŏ A, Nagy N, Olăh J, Kemĕny L, Csőrgő ZB.

Ulcus vulvae acutum Lipschütz in two young female patients. Eur J Dermatol.

2014;24(3):361-4.

8. Sardy M, Wollenberg A, Niedermeier A, Flaig MJ. Genital ulcers associated with Epstein-Barr virus infection (ulcus vulvae acutum). Acta Derm Venereol. 2011;91:55-9.

9. Mourinha V, Costa S, Urzal C, Guerreiro F.

Lipschütz ulcer: Uncommon diagnosis of vulvar ulcerations. BMJ Case Rep. 2016:1-2.

10. Brinca A, Canelas MM, Carvalho MJ, Vieira R, Figueiredo A. Lipschütz ulcer (ulcus vulvae acutum) – A rare cause of genital lesion. An Bras Dermatol. 2012;87(4):622-4.

11. Zouboulis CC. Adamantiades-Behcet disease.

In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. Fitzpatrick’s Dermatology in General Medicine. 8th edition.

New York: Mc Graw Hill; 2012:2033-42.

12. Viera-Baptista P, Silva JL, Beires J, Oliveira JM.

Lipschütz ulcers: Should we rethink this? An analysis of 33 cases. Eur J Obstet Gynecol Reprod Biol. 2016;198:149-52.

13. Edwars L, Lynch PJ. Erosion and Ulcers. In:

Edwards L, Lynch PJ. Genital Dermatology Atlas and Manual. 3th edition. Philadelphia:

Wolters Kluwer; 2017.