Coexistence of vulvar vestibulitis and interstitial cystitis

Abstract

Interstitial cystitis (IC) and vulvar vestibulitis (VVS) are predominantly disorders of adult white women in their reproductive years. Although the etiology and pathogenesis of both conditions are poorly understood, the common embryonic origin of the bladder and vestibule could be important. The coexistence of both conditions in the same patient is uncommon. We present three cases from a gynecology clinic. A 40-year-old white multiparous woman presented with a 9-month history of introital pain and superficial dyspareunia. This was associated with pelvic pain, urinary urgency, burning and hematuria. Antibiotic treatment for suspected ‘recurrent cystitis’ in the past brought no relief. Diagnostic laparoscopy for pelvic pain showed no abnormality. Her vestibule was erythematous and extremely tender when touched with a moistened cotton-tipped swab. Cystoscopy with hydrodistension under general anesthesia revealed diffuse glomerulations. Histology of bladder and vestibular punch biopsies showed abundant chronic inflammatory cells with no dysplasia. Her symptoms improved following a 3-month course of clobetasol propionate 0.05% ointment to the vulvar and six courses of intravesical dimethyl sulfoxide (DMSO). A 28-year-old white nulliparous woman presented with a 14-month history of suprapubic pain, urinary frequency, urgency, introital pain, vulvar soreness and severe superficial dyspareunia. Laparoscopy following continuous oral medroxyprogesterone acetate treatment for mild endometriosis did not show residual pelvic disease. The posterior aspect of the vestibule was erythematous and very tender on swab testing. Cystoscopic and histologic findings confirmed interstitial cystitis. Vestibular punch biopsies revealed numerous chronic inflammatory cells with no evidence of dysplasia or human papilloma virus (HPV) infection. She is still undergoing treatment. Mrs X, a 27-year-old nullipara, was referred with a long history of vulvar burning associated with severe introital dyspareunia, suprapubic pains, urinary frequency, urgency and nocturia. Clinical examination revealed suprapubic tenderness as well as an erythematous and very tender vestibule on swab testing. Cystoscopy following hydrodistension under anesthesia showed diffuse glomerulations. Chronic inflammatory and mast cells were seen in the bladder and vestibular biopsies. There were no atypia. She is currently on intravesical therapy for interstitial cystitis and topical therapy for her vestibulitis. The diagnosis of VVS and IC in this series fulfilled the established clinical criteria (1, 2). Furthermore, the ages of these patients are in consonance with those of Friedrich (3), who first introduced the term vulvar vestibulitis. They also fall within the range of 16–71 years noted by other investigators (4, 5). The documentation of the coexistence of these disorders is important as the search for their etiology continues. Although several mechanisms are being actively investigated, no single causal factor has yet been found that links all the theories. It is conceivable that defective epithelium derived from the embryonic urogenital sinus may underlie the development of both VVS and IC. Further study of this epithelium could provide insight into the pathophysiology of these enigmatic disorders that are important causes of genitourinary pain in Caucasian women.