Lara Salahi
Ashley Winter, MD, remembers the first time she Googled the skin condition lichen sclerosus. Most of the websites listed the autoimmune condition as a rare disease.
In the realm of genital health, some conditions remain shrouded in silence and consequently are more likely to go undercounted and underdiagnosed, Winter, a urologist based in Los Angeles, California, said.
“I truly believe that we just miss the diagnosis a vast majority of the time because there isn’t enough training on [detecting] it,” said Winter.
Lichen sclerosus primarily affects the skin in the genital and anal regions. Estimates of the disease range between 1 in 300 and 1 in 1000 people, according to the US National Institutes of Health. The condition also more commonly occurs among women, and symptoms include hypopigmentation, itching, pain, changes in skin appearance, and skin atrophy.
“Most cases [affect the] genital [area] only, so often patients don’t bring it up because they don’t want to be examined,” said Sarah Lonowski, MD, assistant professor of dermatology and codirector of the Multidisciplinary Autoimmune Skin Disease/Derm-Rheum Program at the University of Nebraska-Lincoln. “It’s a sensitive area, it’s an uncomfortable area to have examined, so it comes with a lot of emotional burden,” for patients, Lonowski said.
photo of Sarah LonowskiSarah Lonowski, MD
Receiving a lichen sclerosus diagnosis can take 5 years or longer, in part because the condition’s symptoms can lead clinicians to first make a diagnosis of a yeast infection or bacterial vaginosis, according to Christina Kraus, MD, assistant professor of dermatology at UCI Health in Irvine, California.
“There is still limited information on this condition in medical education, and it is not uncommon for clinicians who are not in dermatology or gynecology to be unfamiliar with this diagnosis,” Kraus said.
Because no medical tests are available to confirm lichen sclerosus, clinicians diagnose the condition based on the skin’s appearance and symptoms. In some cases, a skin biopsy may help differentiate it from similar rashes that occur in the genital area.
Prepubescent children and postmenopausal women are most likely to develop genital lichen sclerosus, so pediatricians and primary care physicians may be the first to see possible cases, Lonowski said.
Patients “may not mention it unless they’re asked,” Lonowski said, adding clinicians can inquire with patients about genital health, examine bothersome areas, “and refer if you’re not sure.”
Clinicians may also miss the condition during physical exams if they do not examine the vulvar skin, she said. The exact cause also remains elusive, but researchers believe genetic and hormonal factors, as well as an overactive immune response, may contribute to development of the condition.
While lichen sclerosus more frequently occurs in women, men are also affected by the condition. Benjamin N. Breyer, MD, professor and chair of urology at the University of California San Francisco, said lichen sclerosus is one of the most common skin conditions he treats in his male patients.
photo of Benjamin N. BreyerBenjamin N. Breyer, MD
“Advanced cases can cause urethral narrowing, which is a condition I treat commonly,” said Breyer. “Lichen sclerosus is often an underrecognized cause of pain or tearing with erections and sex in men.”
Similar to women, lichen sclerosus presents as white color changes on the skin. For men, the condition can also result in fusion of the shaft skin to the head of the penis and burying or concealment of the penis, Breyer said.
“This leads to challenges with intimacy and urination and can have extensive impacts on quality of life,” said Breyer.
For women, the skin changes often extend to the perianal area and can cause scarring, said Kraus.
“Early scarring may present as adherence of the labia minora to the labia majora or inability to fully retract the clitoral hood from the clitoris,” said Kraus.
In both men and women, lichen sclerosus and another autoimmune condition known as morphea, characterized by skin hardening and discoloration, often present together, said Lonowski.
“If you have a patient with known morphea, it’s important to ask about genital symptoms,” said Lonowski. “The association between the two is fairly strong.”
Circumcision is often the first step to help prevent chronic inflammation among male patients, said Breyer. Because lichen sclerosus is associated with an increased risk for penile cancer, “it is important to biopsy suspicious lesions,” Breyer added.
Increasing awareness of lichen sclerosus is crucial for early detection and timely intervention, said Lonowski. The first-line treatment of genital lichen sclerosus is strong topical steroid ointments to reduce inflammation. Clinicians might prescribe this treatment for use twice daily for 2-3 months and then assesses the patient on their response.
“It is fairly responsive to treatment in most cases,” said Lonowski.
Once symptoms have improved, Lonowski transitions patients to a maintenance regimen, which might include using the same steroid but only three times a week, switching to a topical with a less potent steroid dosage, or using a combination of a topical steroid and a nonsteroidal anti-inflammatory cream. Despite the prolonged use of the drug, she said patients with lichen sclerosus usually do not present with side effects like discoloration or thinning of skin.
“You may achieve control or remission, but we don’t stop treatment completely because we know the natural history of the disease is to have flares and recurrence.”
If left untreated, the condition can lead to atrophy, scarring, and distortion of the genital anatomy and, in some cases, develop into squamous cell carcinoma.
“The fact that you can do a topical cream intervention and prevent cancer is huge,” said Winter.
Winter said open discussions surrounding genital health led by primary care providers can destigmatize conditions like lichen sclerosus and promote early detection and management.
“We need to foster an environment where individuals feel comfortable discussing their symptoms openly,” Winter said. “Increased awareness can pave the way for early detection, which is crucial for managing the condition effectively.”
The experts included in the story reported no relevant disclosures.
Lara Salahi is a freelancer writer living in Massachusetts.
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