Syndrome in question^*

Abstract

Vulvovaginal-gingival syndrome is characterized by erosions and desquamation of the vulva, vagina, and gingiva. We reported a case of a 32-year-old woman presenting with an 8-year history of damage to the vulval and perianal anatomy and limitation of mouth opening. The patient's symptoms were relieved after treatment with topical tacrolimus cream.

Lichen planus; Tacrolimus; Therapeutics

CASE REPORT

A 32-year-old woman presented with an 8-year history of damage to the vulval and perianal anatomy and limitation of mouth opening. Physical examination showed diffuse gingivitis along the inferior gingival mucosa. Asymptomatic white-grey reticulated plaques were detected on the labial-buccal aspect of the maxillary gingivae and the buccal mucosa (Figure 1). There was cutaneous atrophy on the vulval and perianal area. The structures of the labia minora, labia majora and clitoris were lost, with erosions around the vaginal orifice (Figure 2). Scarring of the mucosa of the vaginal orifice and anus manifested as mild stenosis. A biopsy taken from the vulva revealed hyperkeratosis, liquefaction degeneration of the basal cells and inflammatory cells in the upper dermis without atypia (Figure 3).

FIGURE 1
Gingivitis of the inferior gingival mucosa. White-grey reticulated plaques on the buccal mucosa

FIGURE 2
Cutaneous atrophy on the vulval and perianal area, and loss of structure of labia minora, labia majora and clitoris

FIGURE 3
Hyperkeratosis, liquefaction degeneration of the basal cells and inflammatory cells in the upper dermis without atypia (hematoxylin and eosin, × 400)

The patient was treated with topical tacrolimus cream. Erosions improved after 3 weeks.

DISCUSSION

Vulvovaginal-gingival syndrome is a specific type of mucosal lichen planus, characterized by erosions and desquamation of the vulva, vagina, and gingiva.¹ The main sequelae are scar formation, mucous stenosis, impact on specific functions and risk of malignant change.²

Although few cases of vulvovaginal-gingival syndrome have been reported to date, recognizing the syndrome remains a major challenge. Once the mucous stenosis is formed, recovery becomes complicated. Approximately half the patients had been treated repeatedly for presumed vaginal yeast infections by physicians who had misjudged their disease. Several diseases must be considered in the list of differential diagnoses for vulvovaginal-gingival syndrome, including idiopathic desquamative vaginitis, idiopathic erosive vulvitis and vulvar lichen sclerosus. Recognition of desquamative vaginitis syndrome may avoid unnecessary medical and surgical procedures.¹

Early active therapy is required to alleviate symptoms and prevent genital sequelae.³ Topical corticosteroids are the most commonly used drugs for treating the syndrome. Systemic agents used to treat the disorder include corticosteroids, azathioprine, and mycophenolate mofetil.⁴ Recently, topical tacrolimus was shown to be effective.⁵

• Financial Support: None.
• How to cite this article: Wu Y, Qiao J, Fang H. Do you know this syndrome? Vulvovaginal-gingival syndrome. An Bras Dermatol. 2014;89(5):843-4.
• *
  Study carried out at The First Affiliated Hospital, College of Medicine, Zhejiang University - Zhejiang, China.

REFERENCES

• ¹
  Pelisse M. The vulvo-vaginal-gingival syndrome. A new form of erosive lichen planus. Int J Dermatol. 1989;28:381-4.
• ²
  Neill SM, Lewis FM. Vulvovaginal lichen planus: a disease in need of a unified approach. Arch Dermatol. 2008;144:1502-3.
• ³
  Panagiotopoulou N, Wong CS, Winter-Roach B. Vulvovaginal-gingival syndrome. J Obstet Gynaecol. 2010;30:226-30.
• ⁴
  Moutasim KA, Poate TW, Setterfield JF, Challacombe SJ. A case of vulvovaginal gingival lichen planus in association with Good's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008;105:e57-60.
• ⁵
  Cooper SM, Haefner HK, Abrahams-Gessel S, Margesson LJ. Vulvovaginal lichen planus treatment: a survey of current practices. Arch Dermatol. 2008;144:1520-1.

Publication Dates

• Publication in this collection
  Sep-Oct 2014

History

• Received
  02 June 2013
• Accepted
  01 Aug 2013