Multifaceted Approaches for Inflammatory Vulvar Disease

Early-stage inflammatory conditions of the vulva, notably lichen sclerosus or lichen planus, can mimic nonscarring conditions, such as allergic contact dermatitis, making diagnosis challenging before scarring or architectural changes develop, according to Christina Kraus, MD, assistant professor of dermatology at the University of California, Irvine, California.

These autoimmune inflammatory skin conditions mainly affect the genital area, including the vulva and perianal areas.

In a presentation at the 2025 ODAC Dermatology Conference, Kraus outlined strategies for incorporating off-label systemic agents as well as off-label topicals in the management of challenging cases. 

Most patients with vulvar dermatoses will respond to high-potency topical corticosteroids, she said. However, for patients who are not improving with standard of care, cannot tolerate topical steroids, or would benefit from adjunctive therapy, systemic therapies used for other autoimmune conditions can be effective, she said.

For these refractory patients, systemic therapy becomes essential to control inflammation and reduce risks for complications that lead to long-term functional impairment, such as severe scarring, she emphasized. 

Systemic therapies for vulvovaginal lichen planus, which tends involve the mucus membranes and appears as itchy purple lesions, include systemic steroids, 

oral Janus kinase (JAK) inhibitors, cyclosporine, methotrexate, mycophenolate mofetil, hydroxychloroquine, interleukin-23 inhibitors, oral retinoids, apremilast, adalimumab, and intravenous immunoglobin, among other agents, she said.

For patients with lichen sclerosus, which appears as itchy white patches on the vulva, maintenance therapy is important to prevent scarring and reduce the risk for malignancy, Kraus said. The research on systemic therapy for lichen sclerosis includes many of the same agents as lichen planus with the addition of dupilumab, she said. 

Vulvovaginal lichen planus may overlap with lichen sclerosus, and may occur in conjunction with oral, cutaneous, or esophageal lichen planus, she said.

Neoplastic conditions such as extramammary Paget disease, which often presents as a rash with pruritus, can masquerade as inflammatory vulvar dermatosis, Kraus told Medscape Medical News. “If patients suspected of having a vulvar dermatitis do not respond to standard treatments, tissue biopsy and additional evaluations are crucial,” she said. 

“Similarly, unresponsive lesions in lichen sclerosus, such as papules, nodules, or hyperkeratotic areas, should raise suspicion for squamous cell carcinoma or its precursor lesion, differentiated vulvar intraepithelial neoplasia,” she added.

Clinical Considerations

Consider a patient’s estrogen status, as both vulvar lichen sclerosus and vulvovaginal lichen planus are common in postmenopausal women, and addressing these conditions, in addition to the underlying inflammatory disorder, can help with symptom control, Kraus noted.

“Before initiating systemic treatment, shared decision-making with the patient is crucial, including discussions about the off-label nature of these treatments and their risks and benefits,” Kraus told Medscape Medical News. “Even if systemic agents are outside a clinician’s comfort zone, awareness of these options and their application in other inflammatory skin disorders is important,” she said.

Management of inflammatory vulvovaginal conditions in primary care is not unusual, as patients may feel most comfortable disclosing sensitive information to their primary care clinicians, Kraus told Medscape Medical News. In addition, primary care clinicians see patients more frequently and may be more accessible, she said.

However, a patient who is not improving with standard-of-care therapy (usually topical steroids) or requires chronic daily ultrapotent topical steroids without a clear diagnosis, should be referred to someone with vulvar disease expertise (dermatologist or gynecologist), said Kraus. Also, seek specialist referrals for patients who have architectural changes/scarring, severe disease requiring consideration of systemic agents, or non-healing ulcers/erosions/hyperkeratotic lesions that could signal malignancy, she said.

Topical Possibilities

For most patients who are treated with topical agents, off-label options for vulvar lichen sclerosus and vulvovaginal lichen planus include topical calcineurin inhibitors such as tacrolimus. But be sure to counsel patients about the potential for burning with application, Kraus said in her presentation. Off-label topical JAK inhibitors also may be considered; however, there is a pressing need for well-designed studies to evaluate their safety, efficacy, and long-term outcomes in this context, she said.

Significant research gaps remain in the management of vulvovaginal disorders, Kraus told Medscape Medical News. “There are currently no FDA-approved therapies for vulvar lichen sclerosus or vulvovaginal lichen planus, which highlights the need for studies to uncover their molecular pathogenesis, identify biomarkers, and develop targeted therapies,” she said. 

ObGyn Insights

“Vulvar dermatoses, such as vulvar lichen sclerosus and lichen planus, are often unrecognized or misdiagnosed,” said Jill M. Krapf, MD, an obgyn specializing in genitopelvic pain and skin conditions in Tampa, Florida, in an interview. 

“Lichen sclerosus has an average 5-year delay in diagnosis and is often misdiagnosed as chronic yeast infections,” she noted. 

These conditions are diagnosed with a thorough vulvar examination that may include vulvoscopy, said Krapf. If the diagnosis is in question, a vulvar biopsy can confirm and also rule out other conditions, she said. 

The first step, an examination of the vulva, is often rushed or overlooked by clinicians, Krapf told Medscape Medical News.

Tips for Treatment

Identify any coexisting vulvar conditions, such as genitourinary syndrome of menopause, which involves tissue thinning and discomfort, Krapf said. 

Incomplete response to anti-inflammatory topicals for conditions such as lichen sclerosus often stems from lack of instructions on how to most effectively apply the topical medication, she said. “Improper application of topical steroids may lead to skin thinning and superimposed bacterial or yeast infections. If these conditions are ruled out or there is more broad skin involvement in the body, systemic corticosteroids or oral immunosuppressants may be considered,” Krapf noted.

Seeing a doctor with experience and training in vulvar pain and skin conditions is more important than the particular specialty, Krapf told Medscape Medical News. Some gynecologists have advanced training and experience in vulvar dermatoses; some dermatologists examine and treat vulvar dermatoses, while other dermatologists may not, she said. “Refer to a specialist if the diagnosis is unclear after biopsy or if first-line treatments are not working,” said Krapf. “A gynecologist may refer to a dermatologist if there are extragenital skin lesions or if there is a rare skin condition,” she added. 

Kraus received a Dermatology Foundation Career Development Award and disclosed serving as a consultant for Nuvig Therapeutics and LEO Pharma and as an investigator for Incyte Corporation. Krapf had no financial conflicts to disclose. 

Heidi Splete is a journalist in Maryland.