SciELO - Scientific Electronic Library Online

 
vol.67 número1Diagnóstico prenatal de displasia mesenquimal placentariaInversión uterina no puerperal: primer caso reportado en el Perú índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados

Revista

Articulo

Indicadores

  • No hay articulos citadosCitado por SciELO

Links relacionados

  • No hay articulos similaresSimilares en SciELO

Compartir


Revista Peruana de Ginecología y Obstetricia

versión On-line ISSN 2304-5132

Rev. peru. ginecol. obstet. vol.67 no.1 Lima ene./mar 2021

http://dx.doi.org/10.31403/rpgo.v67i2310 

Case Report

Zoon vulvitis in a woman of childbearing age. A case report

Estefanía Ruipérez Pacheco1 

Mar Ramírez Mena1 

Pluvio Coronado Martín2 

1 Facultative specialist in Obstetrics and Gynecology, Institute of Women's Health, Hospital Clínico San Carlos, Madrid, Spain

2 Chief of the Gynecological Oncology Unit, Vulvo-Cervical Pathology, Hospital Clínico San Carlos, Madrid, Spain

ABSTRACT

Zoon vulvitis or vulvitis circumscripta plasmacellularis is a rare chronic inflammatory disease characterized by well-delimited and shiny erythematous plaques that can affect any location of the vulva. It usually occurs in postmenopausal women and poses a difficult differential diagnosis. The need for a vulvar biopsy is emphasized to obtain a certain histological diagnosis. The case of a 36-year-old patient is presented and the current therapeutic options described in the literature are discussed.

Key words: Zoon vulvitis; vulvitis circumscripta plasmacellularis

Introduction

Zoon vulvitis or plasma cell vulvitis is a rare chronic inflammatory disease described for the first time, in 1954, by Garnier and Zoon1,2. It is characterized by well-defined and shiny erythematous plaques that can affect any location of the vulva, in the form of single or multiple plaques. It does not usually affect the vaginal mucosa or other extragenital locations. It can involve the genital mucosa of the vestibule, the paraurethral epithelium, periclitoroid region or the labia minora. The most frequent symptoms are burning, itching, dyspareunia, and dysuria3. It usually occurs in postmenopausal women and poses a difficult differential diagnosis.

The objective of presenting the case is to substantiate the need for a histological study for the diagnosis of this vulvar dermatosis of exceptional observation in clinical practice, as well as to update the best therapeutic option.

Clinical case

We present the case of a 36-year-old woman with two pregnancies and two eutocic deliveries who underwent surgery for a sacrococcygeal cyst. Rest of history without clinical interest.

The patient referred long-standing chronic vulvar itching, burning, discomfort, and dyspareunia. She had consulted on previous occasions for vulvar itching, not responding to topical moisturizers.

The examination revealed a well-defined erythematous plaque in the labia minora and introitus, approximately 1 cm (Figure 1).

Figure 1 erythematous plaque on the labia minora and introitus, well defined. 

The first diagnostic suspicion was vulvar candidiasis, due to the presence of an erythematous plaque. Topical antifungals were prescribed, without obtaining a clinical response.

In the clinical follow-up at one month, given the lack of improvement with topical antifungals, study of the vaginal exudate was performed, which was negative, and a vulvar biopsy (Figures 1 and 2) reported predominantly lymphoplasmacytic vulvitis (Figure 2), corresponding to Zoon vulvitis, an entity characterized by a "pattern of inflammation in the mucosa in relation to chronic dermatoses".

Figure 2 main differences between zoon vulvitis and lichen planus. 

Following diagnosis, topical treatment with high-potency corticosteroids, clobetasol 0.05% cream, three times a day for one month, with subsequent downward regimen, was prescribed twice a day the second month and once a day in the third month. The patient reported a significant clinical improvement in the controls at 3 and 6 months. She is currently asymptomatic, with disappearance of the erythematous plaque.

Discussion

Zoon vulvitis is a benign lesion of the vulva with a low incidence, its equivalent being Zoon balanitis in men1,2.

Presenting symptoms are usually vulvar itching, dyspareunia and dysuria3. On examination, the typical signs are shiny, erythematous plaques, and erosions and telangiectasias may appear on the plaques.

Because the symptoms are nonspecific, it is necessary to make a differential diagnosis (Table 1) with other vulvar dermatoses (lichen planus), as well as with systemic diseases (Behçet’s disease, Chron’s), infections and sexually transmitted diseases (candidiasis, herpes simplex virus, syphilis), precancerous diseases (vulvar intraepithelial neoplasia, Paget's disease) or fixed drug exanthema, being an exclusion diagnosis. Due to the wide range of diseases with which the differential diagnosis must be made, it is mandatory to confirm the diagnosis by biopsy4.

Table 1 Differential diagnosis of plasma cell vulvitis. 

Perhaps due to the similarity in the presentation of the lesions, the main differential diagnosis should be made with lichen planus, an erosive subtype (Figure 2), this being its most frequent form of presentation (70%). It appears as bright erythematous erosions associated with white or Wickham striae, especially on the labia minora and the vestibule, being relatively frequent the involvement of other locations such as the oral mucosa (forming part of the vulvovaginogingival syndrome), the skin or the scalp5,6. Meanwhile, Zoon vulvitis presents with well-defined and shiny erythematous plaques that can affect any location on the vulva in the form of single or multiple plaques. It does not usually affect the vaginal mucosa or other extragenital locations 7. Recently, it has been proposed that to make the diagnosis of erosive lichen planus, the presence of at least three of the nine clinicopathological criteria (Simpson)8) is required: well-defined erythematous areas, hyperkeratotic border and/or Wickham’s striae in the periphery of the lesions, pain/burning, scar changes/loss of normal architecture, vaginal inflammation, involvement of other mucosal surfaces, band inflammatory infiltrate that compromises the dermoepidermal junction, predominantly lymphocytic inflammatory infiltrate, signs of basal epidermal degeneration.

The etiopathogenesis is unknown, although there are different theories (autoimmune, hormonal, irritative).

The definitive diagnosis is histological and is characterized by the presence of a dense inflammatory infiltrate where more than 50% of the cells are plasma cells4 (Figure 2). There is significant endothelial proliferation, with erythrocyte extravasation and hemosiderin deposits. According to the classification of the International Society for the Study of Vulvar Diseases, ISSVD 2006, plasmacellular vulvitis belongs to the vasculopathy pattern8, unlike lichen planus, that corresponds to the lichenoid pattern.

Because the etiology is unknown, multiple different treatments have been chosen, the response to them being inconsistent. The first line of treatment would be high-potency topical steroids such as clobetasol propionate 0.05%, with surgery being the treatment of choice in highly refractory cases9.

Among the medical treatments used we find first-line treatments such as high-potency topical corticosteroids10 and medium-strong topical corticosteroids, second-line treatments such as calcineurin inhibitors, tacrolimus11. Imiquimod, interferon alfa, and even cryotherapy have also been used12. If there is no clinical response to previous treatments, excisional CO2 laser treatment can be used and even excisional treatment in the operating room13.

Given that plasma cell vulvitis is a chronic inflammatory disease and is often associated with periods of remission and relapse, clinical follow-up of the patient is essential.

REFERENCES

1. Zoon JJ. Chronic benign circumscript plasmocytic balanoposthitis. Dermatologica. 1952;105(1):1-7. doi:10.1159/000256880 [ Links ]

2. Garnier G. Benign plasma-cell erythroplasia. Br J Dermatol. 1957;69(3):77-81. doi: 10.1111/j.1365-2133.1957.tb13232.x [ Links ]

3. Martinetti C, Leiro V, Maronna LE, Coringratto M. Vulvitis de Zoon. Dermatol Argent. 2011;17(3):243-5. [ Links ]

4. Virgili A, Levratti A, Marzola A, Corazza M. Retrospective histopathologic reevaluation of 18 cases of plasma cell vulvitis. J Reprod Med. 2005;50(1):3-7. PMID: 15730165 [ Links ]

5. Eisen D. The evaluation of cutaneous, genital, scalp, nail, esophageal, and ocular involvement in patients with oral lichen planus. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999 Oct;88(4):431-6. Doi: 10.1016/s10792104(99)70057-0 [ Links ]

6. Chew A, Stefanato CM, Savarese I, Neill SM, Fenton DA, Lewis FM. Clinical patterns of lichen planopilaris in patients with vulval lichen planus. Br J Dermatol. 2014 Jan;170(1):218-20. Doi: 10.1111/bjd.12609 [ Links ]

7. Virgili A, Corazza M, Minghetti S, Borghi A. Symptoms in plasma cell vulvitis: first observational cohort study on type, frequency and severity. Dermatology. 2015;230(2):113-8 doi: 10.1159/000367786 [ Links ]

8. AEPCC-Guía: Dermatosis inflamatoria de la vulva (liquen escleroso, liquen plano y liquen simple crónico). Publicaciones AEPCC. 2016:1-52. http://www.aepcc.org/aepcc-guiasLinks ]

9. Gurumurthy M, Cairns M, Cruickshank M. Case series of Zoon vulvitis. J Low Genit Tract Dis. 2010;14(1):56-8. doi: 10.1097/LGT.0b013e3181afd524 [ Links ]

10. Botros SM, Dieterich M, Sand PK, Goldberg RP. Successful treatment of Zoon's vulvitis with high potency topical steroid. Int Urogynecol J Pelvic Floor Dysfunct. 2006;17(2):1789. doi: 10.1007/s00192-005-1289-2 [ Links ]

11. Virgili A, Mantovani L, Lauriola MM, Marzola A, Corazza M. Tacrolimus 0.1% ointment: is it really effective in plasma cell vulvitis? Report of four cases. Dermatology. 2008;216(3):2436. doi: 10.1159/000112935 [ Links ]

12. Van Kessel MA, Van Lingen RG, Bovenschen HJ. Vulvitis plasmacellularis circumscripta in pre-existing lichen sclerosus: Treatment with imiquimod 5% cream. J Am Acad Dermatol. 2010:63(1):11-3. DOI: 10.1016/j.jaad.2009.08.018 [ Links ]

13. Toeima E, Sule M, Warren R, Igali L. Diagnosis and treatment of Zoon's vulvitis. J Obstet Gynaecol. 2011;31:473-5. doi: 10.3109/01443615.2011.581317 [ Links ]

There are no sources of funding

The present clinical case was presented at the XXIX Congress of the Spanish Association of Cervical Pathology and Colposcopy - AEPCC, Logroño, Spain, November 23-25, 2017

Cite as: Ruipérez Pacheco E, Ramírez Mena M, Coronado Martín P. Zoon vulvitis in a woman of childbearing age. A case report. Rev Peru Ginecol Obstet. 2021;67(1). DOI: https://doi.org/10.31403/rpgo.v67i2310

Received: August 25, 2020; Accepted: November 03, 2020

There are no conflicts of interest

Creative Commons License Este es un artículo publicado en acceso abierto bajo una licencia Creative Commons