Two Pediatric Cases of Genital Lesion Mimicry
Document Type
Conference Proceeding - Restricted Access
Publication Date
5-9-2025
Abstract
Langerhans Cell Histiocytosis (LCH) is a rare histiocytic disorder involving the proliferation of dendritic cells, also known as Langerhans cells. LCH can present with single organ involvement or multi-system disease. It varies in initial presentation, organ system affected, and severity of condition. Due to its unpredictable characteristics, the diagnosis of LCH is often delayed, even more so in vulvar manifestations, as LCH can mimic many gynecological conditions. The objective of this case report is to bring awareness to the importance of a thorough gynecological evaluation in pediatric patients presenting with vulvar lesions. LCH should remain on the differential for any genital lesion that is not following the expected clinical course.
Patient 1 is a 4-year-old female who initially presented with dysuria and vulvar lesions. The differential included sexually transmitted infections, Lipschütz ulcers, and genital warts. When treatment with Imiquimod and cryotherapy proved ineffective, the patient underwent an excisional biopsy. The pathology results ultimately diagnosed LCH. Her case is representative of focal LCH. Patient 2 is a 7-year-old female who presented with vulvar lesions following a recent upper respiratory infection. The differential diagnosis for her case was similar, however topical treatments for yeast infection and Lipschütz ulcers yielded no improvement. During investigations, the patient developed cervical lymphadenopathy, polydipsia, and shortness of breath. Imaging studies revealed enlarged lymph nodes and an ectopic posterior pituitary. Biopsies of two sites, cervical lymph nodes and vulvar lesions, revealed multisystem LCH.
Patient 1 underwent additional imaging for staging however her diagnosis remained primary Langerhans Cell Histiocytosis of the vulva without multisystem involvement. The patient has experienced no recurrence to date. Patient 2 was started on IV chemotherapy with Cytarabine for the multisystem involvement of LCH. Following four cycles of chemotherapy, the patient reported complete resolution of vulvar lesions. Maintenance chemotherapy was continued with frequent imaging to monitor disease progression. As of when this abstract was written, the patient is doing well and progressing as expected with treatment.
LCH is a clinically complex disease that can result in delayed diagnosis. Its pathogenesis is poorly understood and the clinical course remains variable, with potential for chronic recurrence or fatal outcomes. It is critical for clinicians to consider less common diagnoses when vulvar lesions are not following the expected clinical course and refer as appropriate for further evaluation. Timely diagnosis and intervention is essential for management of LCH.
Recommended Citation
Wiewiora k, Pilarski S, Borse V, McGhee K. Two pediatric cases of genital lesion mimicry. Presented at: Research Day Corewell Health West; 2025 May 9; Grand Rapids, MI.
Comments
2025 Research Day Corewell Health West, Grand Rapids, MI, May 9, 2025. Abstract 1734