Vulvar lichen sclerosus in women of reproductive age

Abstract

Vulvar lichen sclerosus (vLS) is an inflammatory skin condition that predominantly affects the vulvar and perianal regions. Approximately 50% of cases present prior to menopause; however, there is a paucity of data on vLS in women of reproductive age as well as during their pregnancies. A retrospective review was performed at two tertiary referral centers to better describe cases of vLS in women of reproductive age. Thirty-three patients with a mean age of 40 years met inclusion criteria. In this group, vulvar pruritus was the most common presenting symptom (52%); 61% had biopsy-proven vLS, 42% had at least one autoimmune condition, 21% had comorbid depression or anxiety, 33% were given an incorrect diagnosis prior to vLS, and 42% had documented nonadherence to topical steroids. Among the eight patients who became pregnant, four had cesarean deliveries and 63% were symptomatic during pregnancy. When treating a woman who presents with vulvar pruritus or skin changes, vLS should be considered.

Vulvar lichen sclerosus (vLS) is an inflammatory skin condition that affects the vulvar and perianal regions. This disease has predominantly been thought to be diagnosed in either prepubertal or postmenopausal women; however, vLS can develop at any age. In fact, recent studies demonstrate that over 50% of women with vLS have symptom onset before the age of 50.¹ There is a lack of data studying vLS in women of reproductive age, including the effect of pregnancy and delivery on the condition. The aim of this retrospective review was to describe cases of vLS in women of reproductive age, highlight the impact of pregnancy on the disease, and raise awareness of the diagnosis in this age group.

METHODS

We performed a retrospective review of women from Parkland Health and Hospital System and UT Southwestern Medical Center from January 1, 2008, to May 1, 2019. Inclusion criteria included women with an initial diagnosis of vLS between 18 and 45 years of age, an ICD-9 code for lichen sclerosus, and a previous history of pregnancy. Chart review was performed to identify demographic and clinical characteristics. Patients who had a pregnancy after a vLS diagnosis were reviewed further to identify method of delivery, treatment of vLS, and exacerbations of vLS during pregnancy.

RESULTS

Thirty-three women who met the inclusion criteria were identified (Table 1). Their mean age was 40 years, and the mean age at vLS diagnosis was 35 years. Twenty patients (61%) had biopsy-proven vLS and the remainder were clinical diagnoses (39%). No patients had a documented family history of vLS. Vulvar pruritus (52%) was the most common presenting symptom; 42% had one or more autoimmune condition, 21% had comorbid depression or anxiety, 33% were given an incorrect diagnosis prior to vLS, and 42% had documented nonadherence to topical steroids.

Table 1.

Demographic and clinical characteristics of 33 patients with vulvar lichen sclerosus

Characteristic	n (%)
Age (years), mean ±$$SD	39.7 ± 6.3
Age (years) at initial diagnosis, mean ±$$SD	34.5 ± 6.1
Race/ethnicity
Non-Hispanic white	14 (42%)
Hispanic	13 (39%)
Non-Hispanic black	2 (6%)
Unknown	4 (12%)
Initial symptoms
Vulvar pruritus	17 (52%)
Skin changes	14 (42%)
Vulvar pain	7 (21%)
Dyspareunia	5 (15%)
Biopsy proven	20 (61%)
Autoimmune conditions	14 (42%)
Comorbid depression or anxiety	7 (21%)
Family history of autoimmune disorders	12 (36%)
Wrong diagnosis before vLS diagnosis	11 (33%)
Prior oral contraceptive use	12 (36%)
Nonadherence to topical steroids	14 (42%)
Sexual dysfunction due to vLS	10 (30%)

vLS indicates vulvar lichen sclerosus.

Eight of the 33 patients became pregnant after their vLS diagnosis (Table 2). Three had vaginal deliveries, with two complicated by second-degree lacerations. Four patients had cesarean deliveries: three for standard obstetrical reasons and one without documentation of reason. One patient had no record of delivery. In regard to symptoms of vLS during pregnancy, 63% were symptomatic and 25% had exacerbations.

Table 2.

Clinical characteristics of 8 pregnant patients after a vulvar lichen sclerosus diagnosis

Characteristic	N (%)
Gravidity
G1	1 (13%)
G2	4 (50%)
G4	2 (25%)
G6	1 (13%)
Method of delivery
Vaginal	3 (38%)
Cesarean	4 (50%)
Unknown	1 (13%)
vLS treatment during pregnancy
Topical steroids	6 (75%)
Patient discontinued	3 (38%)
None recommended	2 (25%)
vLS symptoms during pregnancy	5 (63%)
Exacerbation during pregnancy	2 (25%)

vLS indicates vulvar lichen sclerosus.

DISCUSSION

Over the last several years, vLS has become increasingly well known. Most patients have been prepubertal and postmenopausal. Our results corroborate associations found within these well-studied groups, including the presence of concomitant autoimmune diseases²: 42% had one or more autoimmune disorder and 36% had a family history in a first-degree relative. At initial presentation, patients with vLS warrant a targeted review of systems to screen for autoimmune conditions associated with vLS, such as thyroid disease.³ Positive findings may necessitate further workup with screening labs.⁴

Consistent with other studies, 52% of patients presented with vulvar pruritus and 42% with architectural changes; less common were pain (21%) and dyspareunia (15%).⁵ The diagnosis of vLS is based on clinical features of depigmentation, texture change, agglutination of labia minora, and ulcerations⁴; therefore, not all patients were biopsied. A biopsy may be necessary if there is a doubt in diagnosis or if patients don’t respond appropriately to treatment. Additionally, untreated vLS can predispose patients to vulvar squamous cell carcinoma; therefore, if patients have indurated or ulcerated painful nodules, a biopsy is warranted.⁶

Patients with vLS have a significant degree of sexual dysfunction and a decreased quality of life.^7,8 Of our patients, 21% had comorbid depression or anxiety. The diagnosis of vLS preceded this diagnosis in three patients. In addition, 30% of women suffered sexual dysfunction due to vLS. Although only one of the patients suffered worsening depression secondary to sexual dysfunction, this is likely underreported,⁹ and several others reported that their sexual dysfunction led to impairments in social functioning. Further attention is necessary to understand the impact vLS has on quality of life and mental health.

Other important factors that arose include incorrect diagnoses and medication adherence. A third of the patients in our cohort were incorrectly diagnosed and treated for recurrent candidiasis and estrogen deficiency at urgent care centers, primary care clinics, and women’s health clinics. Once properly diagnosed, 42% showed noncompliance with medication. Reasons included not refilling medication, discontinuing maintenance therapy, and dislike of topical steroids. Proper treatment and compliance have been shown to improve symptoms and prevent complications.¹⁰ Education on the importance of maintenance therapy, even with resolution of symptoms, is essential.

It has previously been suggested that vLS does not impact pregnancy or mode of delivery.^11–13 Although two patients had lacerations, it is unknown whether this was related to vLS. The majority of patients (63%) were symptomatic during pregnancy, with two exacerbations of vLS (25%). We did find noncompliance in three (38%) and no treatment recommended in two (25%), due to fear of impacting pregnancy. Therefore, it is crucial we recommend continued therapy to patients, as topical corticosteroids are safe and efficacious during pregnancy.¹⁴

In conclusion, vLS should be considered in patients of all ages, including reproductive-age women, when faced with vulvar pruritus or skin changes, especially with concurrent autoimmune disorders. vLS can occur during pregnancy and potentially affect pregnancy.