Vulvovaginal Involvement in Netherton Syndrome: A Case Report
January 2024
in “
JAAD case reports
”
Netherton syndrome genodermatosis skin desquamation hair shaft anomalies immune system disturbances erythematous plaques antibacterial treatments antifungal treatments topical corticosteroids emollients oral contraceptives scaly erythroderma atopic dermatitis skin peeling hair abnormalities immune issues red plaques antibacterial antifungal steroid creams moisturizers birth control pills scaly skin eczema
TLDR Netherton syndrome can cause severe and chronic vulvovaginal symptoms that may improve with continuous oral contraceptives.
Netherton syndrome (NS) is a rare genodermatosis affecting approximately 1 in 200,000 individuals, characterized by skin desquamation, hair shaft anomalies, and immune system disturbances. This case report discusses a 27-year-old woman with NS who presented with chronic and severe vulvovaginal symptoms, including widespread erythematous plaques and significant white discharge. Various treatments were attempted, including systemic and topical antibacterial and antifungal treatments, topical corticosteroids, emollients, and oral contraceptives. The patient's condition improved, but still flares premenstrually and following sun exposure. Continuous oral contraceptives provided significant relief for the patient. The report highlights the importance of considering NS in patients with hair shaft anomalies and profound scaly erythroderma that does not respond to usual atopic dermatitis treatment, including vulvovaginal areas.
