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Vulvovaginal Disease

Vestibulovaginal Sclerosis in a Transgender Man on Testosterone

O'Sullivan, Clare MBBS1; Day, Tania MD, PhD1,2; Scurry, James FRCPA2,3

Author Information
Journal of Lower Genital Tract Disease: April 2020 - Volume 24 - Issue 2 - p 229-231
doi: 10.1097/LGT.0000000000000510
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Vestibulovaginal sclerosis (VVS) presents as a white plaque in the vestibule or vagina, previously reported only in perimenopausal and postmenopausal women1–3 (see Table 1). This case expands the clinicopathologic definition of VVS: it may occur during reproductive age and the sclerosis may be focal. This case also highlights a knowledge gap regarding the impact of gender-affirming testosterone on vulvovaginal disease.

TABLE 1
TABLE 1:
Summary of Published Cases of VVS

CASE REPORT

A 26-year-old transgender man presented with a painful white vulvar plaque present for 4 months. He denied injury, systemic illness, travel, change in medications, or traumatic sexual practices. Comorbidities included low back pain, pelvic pain with recent negative laparoscopy, restless leg syndrome, anxiety, bipolar disorder, and previous uncomplicated appendectomy. His medications were depot testosterone enanthate 125 mg every 10 days for 2 years, sodium valproate, escitalopram, and quetiapine. He was amenorrheic for 14 months, and cervical screening was normal and up-to-date. He received school-based quadrivalent human papillomavirus (HPV) vaccination. He smoked tobacco, used no other substances, and had a supportive relationship with a woman.

Vulvar examination demonstrated clitoromegaly and a white plaque over most of the hymenal ring that was tender to light palpation (see Figure 1). Cotton swab testing revealed scores of 7 to 9 over the abnormal skin, with a score at normal skin of 2. He consented to 4-mm punch biopsy of the hymen at 8 o'clock. Internal examination showed tight, tender levator ani muscles, and a mobile, anteverted, non-tender uterus.

FIGURE 1
FIGURE 1:
Clitoromegaly with otherwise normal vulvar architecture, and a white plaque extending over most of the hymenal ring.

The initial impression was chronic pelvic pain and vulvodynia with multiple associated factors. The etiology and clinical impact of the white plaque was unclear. The care plan included oral gabapentin, pelvic floor physiotherapy, and engagement with endocrinology, the pain team, and psychiatry.

Biopsy showed squamous epithelium with marked hyperkeratosis, hypergranulosis, irregular acanthosis, normal basal cell layer, absent lymphocytic infiltrate, and negative p16 immunostaining. The histopathologic diagnosis was benign hyperkeratosis and cannot exclude condyloma.

One month later, the lesion was unchanged. He had not accessed physiotherapy and took gabapentin 300 mg at night. He requested excision and was advised that this may not improve symptoms, but the larger specimen might provide a diagnosis. He underwent a posterior vestibulectomy with vaginal advancement, modified to remove the entire white plaque but leave normal skin in situ. For postoperative analgesia, a pudendal block with 0.25% bupivacaine was administered at the sacrospinous ligaments with 10 mL on each side.

The histopathology was consistent with vestibular sclerosis (see Figure 2). The hematoxylin and eosin stain demonstrated hyperkeratotic and acanthotic epithelium with a normal basal layer, sparse lymphocytic infiltrate, and focal sclerosis of the lamina propria. Periodic acid–Schiff (PAS) staining highlighted a thick basement membrane and focal subepithelial sclerosis. The specimen's negative HPV status was confirmed at a research laboratory with an L1 primer that detects any HPV type.

FIGURE 2
FIGURE 2:
Vestibulectomy specimen showing (A) hyperkeratosis, irregular acanthosis, normal basal layer, thickened basement membrane, a focal thin band of sclerosis, and sparse lymphocytic infiltrate, hematoxylin and eosin, ×100, and (B) PAS stain highlighting the thick basement membrane and subepithelial sclerosis, ×100.

Three months after procedure, the surgical site was well healed with cotton swab scores of 0 to 2. Pudendal block provided 8 weeks of relief, followed by gradual return of myofascial pain. With time and multidisciplinary management, he reported intermittent pain-free periods. There was no VVS recurrence during 20 months of follow-up.

DISCUSSION

The incidence, etiology, and natural history of VVS are unknown.1–3 Estrogen deficiency and trauma are proposed risk factors, but these are not universal across cases. The clinicopathologic features of VVS are (a) well-demarcated white plaque in the vestibule and/or vagina, sometimes incorporating a fissure, (b) subepithelial sclerosis, (c) absent or sparse lymphocytic infiltrate, and (d) normal or nonspecific epithelial appearance. Keratinization may occur but is not a diagnostic requirement.2,3 This case is unusual for clinical and histopathologic reasons: VVS occurred in a young transman on gender-affirming testosterone, and the focal distribution of sclerosis meant that punch biopsy was nondiagnostic.

The differential diagnosis for a white vestibular plaque includes condyloma, high-grade squamous intraepithelial lesion, lichen sclerosus (LS), and epidermolytic hyperkeratosis.2,4 The appearance was not typical for HPV-related disease, also excluded by negative p16 and HPV DNA. Mucosal LS is usually contiguous with vulvar disease; there was also no pruritus, no other dermatosis, and no lichenoid tissue reaction.2 Epidermolytic hyperkeratosis is poorly understood, infrequently reported on the vulva, and has a benign clinical trajectory.

The role of VVS in his pain syndrome is unclear. The 2015 classification of vulvodynia emphasizes assessment for associated factors, to include hormonal status, musculoskeletal involvement, neurologic mechanisms, and psychosocial factors, each representing a potential treatment target.5 Little is known about chronic pain in transmen. A study of transgender US Medicare beneficiaries found more claims for fibromyalgia and migraine compared with cisgender patients, with rates nearly doubled in those insured because of age rather than disability.6 The impact of high-dose testosterone on vulvovaginal disease is unknown, but estrogen deficiency may alter local mucosal immune function and steroid receptor expression, potentially enhancing vestibular nociception.7 Supporting the association between exogenous androgens and pain, a survey study of transmen identified that half reported pain with penetration while on testosterone, in contrast to rates of 20% before initiation of gender-affirming hormones.8

Excision is the only treatment reported as effective in series of VVS. In this case, diagnostic posterior vestibulectomy allowed for identification of focal sclerosis and comprehensive histopathologic assessment to exclude LS. It also yielded local symptom resolution, but pain at other sites only improved after persistent engagement with neuromodulators, physiotherapy, and psychological modalities.

REFERENCES

1. Fadare O. Vaginal stromal sclerosis: a distinctive stromal change associated with vaginal atrophy. Int J Gynaecol Pathol 2011;30:295–300.
2. Day T, Burston K, Dennerstein G, et al. Vestibulovaginal sclerosis versus lichen sclerosus. Int J Gynaecol Pathol 2018;37:356–63.
3. Croker BA, Scurry JP, Petry FM, et al. Vestibular sclerosis: is this a new, distinct clinicopathological entity? J Low Genit Tract Dis 2018;22:260–3.
4. Iglesias-Plaza A, Del Alcazar-Viladomiu W, Paret-Sanz C, et al. Asymptomatic hyperkeratotic plaque on the vulva of a patient with lichen sclerosus. Clin Exp Dermatol 2018;43:620–2.
5. Bornstein J, Goldstein AT, Stockdale CK, et al. 2015 ISSVD, ISSWSH, and IPPS consensus terminology and classification of persistent vulvar pain and vulvodynia. J Low Genit Tract Dis 2016;20:126–30.
6. Dragon C, Guerino P, Ewald E, et al. Transgender Medicare beneficiaries and chronic conditions: exploring fee-for-service claims data. LGBT Health 2017;4:404–11.
7. Leclair CM, Goetsch MF, Li H, et al. Histopathologic characteristics of menopausal vestibulodynia. Obstet Gynaecol 2013;122:787–9.
8. Ribbink PJA, Goetsch MF, Garg B. Trans men using testosterone have a high prevalence of penetrative vestibular pain. J Low Genit Tract Dis 2019;23:S56.
Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the ASCCP.