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Case Reports

Abstract

Vulvar hypopigmentation in pediatric patients can result from various inflammatory and autoimmune dermatoses. lichen sclerosus (LS) and vitiligo, often present with overlapping clinical and histologic features. We report the case of a 5-year-old girl who presented with vulvar itching, burning, and progressive hypopigmentation involving the labia, perianal region, and lower abdomen. A vulvar biopsy revealed lichenoid interface dermatitis, lymphoplasmacytic infiltration, and homogenized collagen, findings consistent with LS. However, SOX10 immunostaining demonstrated loss of melanocytes, raising concern for concurrent vitiligo. This case highlights the diagnostic complexity of distinguishing LS from vitiligo, especially since LS can present with vitiligo-like depigmentation, even in the absence of surface textural changes. Both conditions share a common pathogenetic mechanism, interface dermatitis, which contributes to depigmentation. However, differentiating between them histologically and immunohistochemically remains a challenge, due to the limited literature directly comparing these features. In our case, further clinical investigation, along with the presence of extragenital segmental abdominal vitiligo, supported the possibility of coexisting anogenital vitiligo alongside LS. This overlap raises important diagnostic questions, as the coexistence of two depigmenting disorders within a localized area may reflect a broader spectrum of a single disease process, rather than two distinct conditions. To address this diagnostic dilemma, a thorough literature review was conducted in search of more quantitative markers or criteria to aid in distinguishing between these entities. Notably, the patient also exhibited signs of precocious puberty, the cause of which remains undetermined. The possible role of shared autoimmune or hormonal pathways in the pathogenesis warrants further investigation.