Verruciform xanthoma (VX) is a rare benign mucocutaneous proliferative lesion that primarily affects the oral mucosa and, in rare cases, the genital mucosa and skin. Due to its uncommon occurrence and varied, non-specific clinical manifestations, the condition is prone to misdiagnosis and missed diagnosis. This report introduces an even rarer case of pediatric vulvar VX, providing a detailed interpretation and analysis of the ultrasonographic features of this condition. Furthermore, in conjunction with previous literature, this report summarizes and analyzes the currently reported vulvar VX cases, aiming to obtain effective diagnostic and therapeutic information, enhance preoperative diagnostic accuracy, and improve patient prognosis.

Vulvar lymphangioma circumscriptum is a rare, benign lymphatic malformation that can mimic infectious or neoplastic lesions, often leading to delayed diagnoses. This report presents the case of a 48-year-old woman who presented with a 10-year history of wart-like lesions on the vulva, causing soreness, pruritus, and an impaired quality of life. Examination revealed multiple fluid-filled papules and swelling of the left labia majora. She underwent surgical excision, and histopathology confirmed lymphangioma circumscriptum without evidence of malignancy. At 12-month follow-up, she remained asymptomatic with no recurrence. This case highlights the importance of considering lymphangioma circumscriptum in chronic vulvar lesions and demonstrates that surgical excision can provide effective symptom relief.

Buschke-Löwenstein tumor (BLT), also known as giant condyloma acuminatum, is an uncommon manifestation of low-risk human papillomavirus (HPV) infection, characterized by massive exophytic growth and substantial local morbidity. Although histologically low-grade, BLT can become profoundly function-limiting and carries a variable risk of malignant transformation, particularly in long-standing or untreated lesions. We report a case of a neglected giant vulvar BLT in an adult woman who presented after several years of progressive growth, resulting in impaired ambulation, hygiene difficulties, and sexual dysfunction. Surgical excision with margin control was performed, leading to functional improvement, and histopathological examination confirmed BLT, without evidence of invasive squamous cell carcinoma (SCC). This case highlights the clinical consequences of delayed diagnosis and limited access to care, positioning BLT as a paradigmatic clinically neglected disease. Emerging pathogenetic evidence supports the concept that BLT represents an extreme along a continuous spectrum of low-risk HPV-associated disease, in which sustained epithelial hyperplasia, stochastic persistence of infected basal cell clones, and local immune modulation permit progressive, space-occupying growth over time. Early recognition and definitive surgical management remain essential to prevent severe morbidity and reduce the risk of recurrence or malignant progression.

Background: Treatment options for vulvar lichen sclerosus (VLS) remain limited; therefore, therapies that improve quality of life and reduce neoplastic risk are needed. Photodynamic therapy (PDT) is a potential option. This study aimed to evaluate quality of life and sexual function in patients treated according to the protocol used at our institution. Methods: Forty patients with refractory VLS underwent PDT using a 10% 5-aminolevulinic acid nanoemulsion (Ameluz[®]) applied to lesions under an occlusive aluminum foil dressing. Patients received 1-6 sessions of 10 min illumination (LED: 37 J/cm[2], ~77 mW/cm[2]) at 4-6-week intervals. The Dermatology Life Quality Index (DLQI) and Female Sexual Function Index (FSFI) were used for assessment. Results: Thirty-seven participants answered DLQI, while 20 declared themselves to be sexually active and were included in the analysis. Greater number of PDT sessions was associated with a lower DLQI score (τ = -0.583; adjusted p < 0.001). The number of PDT sessions and the total FSFI score (p = 0.014), as well as desire (p = 0.016), arousal (p = 0.020), orgasm (p = 0.020), and satisfaction (p = 0.016) domains were significantly correlated. Age correlated positively with DLQI scores (p = 0.016), indicating greater disease burden in older patients. Longer disease duration was also associated with poorer quality of life (p = 0.020). Conclusions: PDT can be considered an effective treatment for patients with VLS refractory to standard topical corticosteroid and calcineurin inhibitor therapies when delivered using a refined, patient-centered protocol. This optimized approach used in our institution is based on short irradiation time and precise light delivery, providing a favorable balance between therapeutic efficacy, patient comfort, and treatment feasibility. Our findings also suggest that the cumulative number of PDT sessions is a key factor for clinical response. Further studies should address long-term outcomes.

Papillary immature metaplasia, originally described in the uterine cervix, is increasingly recognized as a distinctive squamous intraepithelial lesion rather than a true metaplastic process. Accordingly, the term papillary squamous intraepithelial lesion (PSIL) has been proposed. PSIL occurring outside the uterine cervix is exceedingly rare. In this study, we characterize the clinicopathologic, immunophenotypic, and virologic features of 25 cases of PSIL involving non-cervical sites (vagina, vulva, anus, and endometrium). All lesions demonstrated exophytic papillary architecture lined by uniform immature squamous cells with limited surface maturation. Mitotic figures were frequently identified in the lower half of the epithelium. Low-risk HPV E6/E7 mRNA was detected in 76% of cases, whereas none harbored high-risk HPV. HPV transcripts exhibited a distinctive punctate distribution throughout the epithelium, contrasting with the superficial clustered pattern seen in condyloma/LSIL. Non-block p16 immunostaining pattern was seen in all PSILs, while Ki-67 labeling indices were increased (median 50%), predominantly involving the lower half to two-thirds of the epithelium. Clinical follow-up (median 32 months) demonstrated recurrence in 43% of cases, most commonly with incomplete excision. Our study shows that non-cervical PSIL shares morphologic, immunophenotypic, and virologic features with cervical PSIL. Despite the absence of block-type p16 expression, PSIL demonstrates high proliferative activity, frequently leading to diagnostic confusion with HSIL. Given its distinctive papillary architecture, unusual HPV distribution pattern, and tendency for recurrence, PSIL represents a unique low-risk HPV-associated intraepithelial lesion distinct from conventional LSIL and HSIL. Adoption of the term papillary squamous intraepithelial lesion is recommended in accordance with LAST terminology.

Background and Objectives: Synchronous cervical and vulvar squamous intraepithelial lesions are rarely reported. In most cases, these lesions are associated with high-risk human papillomavirus (HPV) infection and follow the conventional HPV-related pathway. Rarely, vulvar lesions may show an unusual immunohistochemical profile, with block-type p16 expression accompanied by aberrant p53 staining, creating diagnostic and etiopathogenetic challenges. Case Presentation: We report the case of an 83-year-old woman who presented with metrorrhagia and a symptomatic vulvar lesion. Histopathological evaluation revealed synchronous high-grade squamous intraepithelial lesion of the cervix and vulvar high-grade squamous intraepithelial lesion (VIN 3). Immunohistochemically, the cervical lesion showed block-type p16 positivity and a wild-type p53 pattern, supporting a conventional HPV-associated profile. In contrast, the vulvar lesion also demonstrated block-type p16 positivity, but with aberrant p53 overexpression, representing an unusual double-positive immunophenotype. Conclusions: This case highlights a rare presentation of synchronous lower genital tract squamous intraepithelial lesions with divergent immunophenotypic features. Accurate interpretation requires integration of morphology and immunohistochemistry, while the absence of direct HPV testing and TP53 molecular analysis limits definitive etiopathogenetic classification. Reporting such cases may broaden awareness of unusual vulvar precursor lesions and potential diagnostic pitfalls in routine practice.

Discoid lupus erythematosus (DLE) is a chronic cutaneous manifestation of lupus that may occur independently or precede systemic disease. Vulvar involvement is exceptionally rare and can mimic more common vulvar dermatoses. We report a case of vulvar hypertrophic DLE in a 55-year-old African-American woman with systemic lupus erythematosus who was presented with an asymptomatic erythematous, ulcerated vulvar lesion initially suspected to represent herpes simplex virus infection or lichen sclerosus. Histopathologic examination demonstrated vacuolar interface dermatitis, follicular plugging, basement membrane thickening, and stromal mucin deposition, findings consistent with DLE. The lesion developed during a lupus flare and lacked the pruritus typical of lichen sclerosus. Management included continuation of immunosuppressive therapy with clinical improvement and residual postinflammatory hypopigmentation. This case highlights the importance of considering lupus-related disease in the differential diagnosis of atypical vulvar lesions and underscores the role of biopsy in establishing an accurate diagnosis.

Cutaneous tuberculosis is an uncommon extrapulmonary manifestation of Mycobacterium tuberculosis infection. Vulvar involvement is an exceptionally rare clinical entity that poses significant diagnostic challenges due to its similarity to other dermatologic and gynecologic conditions. We present the case of a 25-year-old immunocompetent woman with an 18-month history of a vulvar and perineal lesion that developed over the scar of a previous episiotomy. The patient was initially managed for a recurrent bacterial soft-tissue infection and received multiple antibiotic regimens without a therapeutic response. Upon admission, a raised, erythematous, granulomatous plaque with fistulization was observed. Definitive diagnosis was established through histopathological examination, which revealed chronic granulomatous inflammation and a Ziehl-Neelsen stain positive for acid-fast bacilli. To further complement the diagnostic workup, an interferon-gamma release assay was performed and was reactive; the tuberculin skin test was deferred due to the patient's prior BCG vaccination. Standard four-drug antituberculous therapy was initiated, resulting in a favorable clinical evolution. Vulvar tuberculosis should be considered in the differential diagnosis of chronic, ulcerated, or granulomatous genital lesions unresponsive to conventional antibiotic therapy, particularly in patients from endemic areas. Early clinical suspicion and timely biopsy are crucial to prevent morbidity associated with delayed diagnosis.