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Lipschütz ulcer (also known as ulcus vulvae acutum) is a rare, painful genital ulceration typically occurring in adolescent girls or young women, often in association with a systemic illness like a viral infection. It's non-sexually transmitted and usually self-limited.

🔍 Connection to PFAPA Syndrome:

While not classically described as part of PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis), recent case reports suggest that Lipschütz-like ulcers may occasionally occur in PFAPA, particularly during flares. This association is rare but noteworthy.

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📌 Quick Pediatric Pearl: Lipschütz Ulcers in PFAPA

🧠 What is it?
A non-sexually transmitted, painful vulvar ulcer that may rarely appear in PFAPA flares.

📅 When to suspect?

• Acute, painful genital ulcer in a young girl

• Associated fever and systemic symptoms

• Negative for common STIs

• History of periodic fevers or other PFAPA features

🧬 Pathophysiology:
Thought to be immune-mediated or reactive to infection/inflammation.

🔎 Diagnosis:

• Clinical + exclusion of other causes (HSV, Behçet’s, etc.)

• Consider PFAPA if recurrent and associated with pharyngitis/aphthae/cervical adenitis

💊 Treatment:

• Supportive: analgesia, hygiene, topical anesthetics

• PFAPA management: steroids during flare, colchicine for prevention

🩺 Pearl:
Lipschütz ulcers are a rare but important clue in recurrent febrile illnesses like PFAPA. Don't mistake them for STIs—especially in prepubertal girls.

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