Turner syndrome: don't forget the vulva

Area of Science:

• Gynecology
• Autoimmune Diseases
• Oncology

Background:

• Turner syndrome (TS) is associated with an increased prevalence of autoimmune conditions, including lichen sclerosus (LS), estimated at 17%.
• Lichen sclerosus (LS) is a recognized precursor to the development of vulvar cancer.
• Vulvar cancer risk is a significant concern in women with TS and LS.

Purpose of the Study:

• To highlight the association between Turner syndrome, lichen sclerosus, and vulvar cancer.
• To emphasize the importance of regular vulvar screening in adult women with TS.
• To present a case of vulvar cancer in a patient with a history suggestive of LS.

Main Methods:

• Case presentation of a 44-year-old woman with vulvar cancer and underlying lichen sclerosus.
• Review of the known link between TS and autoimmune conditions like LS.
• Discussion of clinical presentation, histology, and management recommendations.

Main Results:

• The case confirmed squamous cell carcinoma with underlying LS in a patient with a history of pruritus.
• LS is a known precursor to vulvar cancer, and TS patients have an increased risk of developing LS.
• Early detection and monitoring are crucial for managing LS and preventing vulvar cancer progression.

Conclusions:

• Patients with Turner syndrome (TS) are at increased risk for developing lichen sclerosus (LS).
• Lichen sclerosus (LS) is a known precursor to vulvar cancer.
• Annual vulvar screening and patient awareness of symptoms are recommended for TS women with LS to mitigate vulvar cancer risk.