Genital Ulcers A Rare Symptom of Primary Sjögren’s Syndrome, Case Report Suggests

Researchers have identified genital ulcers as an initial symptom of primary Sjögren’s syndrome for the first time in a recent case report.

The report, “Lipschütz Genital Ulceration as Initial Manifestation of Primary Sjögren’s Syndrome,” appeared in Case Reports in Obstetrics and Gynecology.

Women affected by primary Sjögren’s syndrome may experience some gynecological symptoms such as dryness, painful sexual intercourse, or rashes. But to date, genital ulcers have not been described as a symptom of the disease.

Researchers in Portugal have now described the case of a 26-year-old woman who presented with ulcers, or sores, in her vulva — the outer part of the female reproductive system.

The patient went to the emergency room complaining of pain in the back of her eyes, painful swallowing, fever, and vaginal discomfort. Her genital ulcers had failed to improve with anti-inflammatory and herpes medication, prescribed at a primary healthcare facility.

Two days after her initial evaluation, the patient complained of increasing vulvar pain. A clinical examination found liquid accumulation in the vulva, with ulcers on the labia minora and majora, vagina, and cervix. Her groin lymph nodes were also swollen.

Blood tests revealed signs of inflammation, but the patient was negative for all infections that could be causing the symptoms — including the herpes virus, Ebstein-Barr virus, rubella, hepatitis, human immunodeficiency virus, and syphilis.

She also had not used any other medication or had sexual activity in the past six months, ruling out the possibility of an acquired sexual infection.

Her family had no history of autoimmune diseases, but her father had experienced some episodes of oral ulcers. When asked directly, the woman confirmed she had also experienced mild eye dryness and frequent dry mouth discomfort.

At this point, a detailed blood analysis was conducted, which was positive for rheumatoid factor, anti-nuclear antibodies, and SSA/Ro antibodies, suggesting an autoimmune disorder. A biopsy from the labial salivary gland showed immune cell infiltration, leading to a diagnosis of Sjögren’s syndrome.

The patient started treatment with 20 mg of oral prednisolone per day. Within two weeks, all her vulva manifestations resolved, but she lost part of her left labia.

“Reactive acute genital ulcers (Lipschütz ulcers) should be considered as a possible manifestation of many autoimmune/inflammatory disorders, beyond the classic associations such as Behcet’s syndrome or Crohn’s disease,” the researchers concluded.