The most common presenting symptoms of vulvar lichen sclerosus (LS) in the pediatric age group are pruritis and vulvovaginal irritation. Here we describe an atypical first presentation of LS, characterized by a periclitoral keratin pearl.

A 6 year 2 month old patient was referred to a tertiary pediatric and adolescent gynecology (PAG) clinic for a periclitoral cyst which had been present for at least several months. The patient's mother had noted occasional extrusion of white material, with no change in cyst size. The patient described pain with wiping or cleaning the vulvar area. On examination, the labial architecture was normal and there were no skin lesions. To the left of the clitoral hood, a raised area was noted (Figure 1) and a small amount of white material extruded. After application of 1% lidocaine gel and with retraction of the clitoral hood, a 0.75 × 0.5cm keratin pearl was removed (Figure 2). Daily sitz baths were recommended. At follow up four months later, the patient was doing well with no itching or pain, and no bladder or bowel symptoms. On examination, thick white material was again noted extruding from the same area. A small midline clitoral hood adhesion, posterior to the glans, was visualized which created the space for the pseudocyst. Pallor of the medial labia majora with mild thinning and wrinkling of the skin was also seen, consistent with early LS. The patient was prescribed topical clobetasol for treatment of LS with clitoral adhesions.

Keratin pearls form from the buildup of secretions and debris in a pseudocystic space, such as that formed by clitoral adhesions. Symptoms include pain with friction and a foreign body sensation[1]. Surgical treatment of clitoral adhesions causing keratin pearls has previously been described[2]. In the pediatric population, topical estrogen has been successfully used to non-operatively treat clitoral adhesions and prevent keratin pearl recurrence[1]. In adult women presenting with clitoral adhesions with or without keratin pearls, 6-17% have a history of lichen sclerosus[3,4]. As described in this case report, the PAG provider should consider LS in the differential diagnosis of clitoral adhesions causing keratin pearls. Serial examinations provide the opportunity to identify early skin changes consistent with LS, and to implement topical steroids for treatment of LS and prevention of keratin pearl formation. 1. Bragiel et al. JPAG (2022) j.jpag.2022.10.002. 2. King and Goldstein. Sexual Medicine (2017) j.jsxm.2017.04.052. 3. Krapf J et al. Sexual Medicine (2022) j.jsxm.2022.05.032. 4. Aerts L et al. Sexual Medicine (2018) j.esxm.2018.01.003.

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