Langerhans Cell Histiocytosis of Vulva in Pediatric Patient

Document Type

Conference Proceeding

Publication Date

4-2025

Publication Title

Journal of Pediatric and Adolescent Gynecology

Abstract

Background: Langerhans Cell Histiocytosis (LCH) is a rare disorder characterized by proliferation of histiocytes in various tissues, most commonly affecting the bone and skin. Its clinical manifestations vary widely, ranging from local lesions to disseminated involvement. Patient presentations range from self-healing skin lesions to multi-organ dysfunction resistant to treatment. The unpredictable presentation of LCH can lead to diagnostic delay, particularly in pediatric patients with vulvar lesions at presentation. This case report highlights the significance of a proper gynecological evaluation in pediatric patients, including biopsy and specialist referral if the etiology remains unclear.

Case: A 4-year-old female initially presented with dysuria and four vulvar lesions. An initial evaluation included testing for sexually transmitted infections which returned negative. Despite empiric treatment for a suspected urinary tract infection, her symptoms persisted, prompting a referral to Dermatology who diagnosed the patient with genital warts. When treatment with Imiquimod and cryotherapy proved ineffective, a self-referral to Pediatric and Adolescent Gynecology was made by the patient's parents. A pelvic exam under anesthesia revealed 10 small, round, flesh-colored papules, several with central umbilication, located on the right labia majora. These lesions were removed via excisional biopsy and pathology results diagnosed Langerhans Cell Histiocytosis. Additional imaging for staging purposes classified the diagnosis as primary Langerhans Cell Histiocytosis of the vulva without multisystem involvement. The patient has experienced no recurrence to date.

Comments: Primary Langerhans Cell Histiocytosis of the vulva in a pediatric patient is rare, with only two cases reported in scientific literature to date. This case illustrates the importance of a thorough gynecological evaluation in a pediatric patient presenting with vulvar lesions. Despite its rarity, clinicians should maintain a high index of suspicion for Langerhans Cell Histiocytosis if the clinical course of a previously made diagnosis is not progressing as suspected. Early diagnosis and prompt intervention are crucial for effective disease management in this condition.

Volume

38

Issue

2

First Page

259

Comments

NASPAG Annual Clinical Research Meeting, April 3-5, 2025, Toronto, ON, Canada

Last Page

259

DOI

10.1016/j.jpag.2025.01.095

ISSN

1083-3188

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