Vulvovaginal graft-versus-host disease (GVHD) is an underdiagnosed and poorly recognized complication of hematopoietic stem cell transplantation (HSCT). Previous studies have reported findings restricted to predominantly adult populations. We report a case series of pediatric and young adult vulvovaginal GVHD, which was identified in 19 patients (median age, 11.8 years; range, 2.4 to 21.9 years) out of a total 302 female patients who underwent transplantation over an 8-year period at a pediatric HSCT center. The majority of patients had concomitant nongenital GVHD; only 1 patient had isolated vulvovaginal GVHD. The median time from bone marrow transplantation to diagnosis of vulvovaginal GVHD was 30 months (range, 2.3 to 97.5 months). A high percentage of the patients in our series were without vulvar or vaginal symptoms (n = 8; 42%), even though 17 patients (89%) presented with grade 3 disease based on current adult grading scales. Vulvar examination findings most frequently included interlabial and clitoral hood adhesions (89%), loss of architecture of the labia minora or clitoral hood (42%), and skin erosions or fissures (37%). Only 5 patients underwent a speculum exam, none of whom had vaginal GVHD. Examination findings of primary ovarian insufficiency (POI) can overlap with those of GVHD, and 6 patients (32%) in our cohort were diagnosed with POI. Only 1 patient was on systemic hormone replacement therapy at the time of vulvovaginal GVHD diagnosis. The majority of patients (n = 16) were treated with topical steroid therapy, with a median time to response of 43 days. Five patients (26%) had a complete response to therapy, and 10 patients (53%) had a partial response. This case series provides valuable insight into pediatric and young adult vulvovaginal GVHD and highlights the need for increased screening for vulvar disease in this population.

中文翻译：

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小儿和年轻成人外阴阴道移植物抗宿主病。

外阴阴道移植物抗宿主病（GVHD）是造血干细胞移植（HSCT）的一种未被充分诊断和认识不足的并发症。先前的研究已报告发现仅限于成年人口。我们报告了一个小儿和年轻成人外阴阴道GVHD病例系列，在302例在8年内接受移植的女性患者中，有19例（中位年龄为11.8岁；范围为2.4至21.9岁）被确定。儿科HSCT中心。大多数患者伴有非生殖器GVHD。仅1例患者分离出阴道阴道GVHD。从骨髓移植到诊断出阴道阴道GVHD的中位时间为30个月（范围2.3至97.5个月）。在我们的系列中，有很大比例的患者没有外阴或阴道症状（n = 8； 42％），即使根据目前的成人分级量表，有17名患者（89％）患有3级疾病。外阴检查结果最常见的包括阴唇和阴蒂之间的粘连（89％），小阴唇或阴蒂之间的结构丧失（42％）以及皮肤糜烂或裂痕（37％）。仅5名患者接受了窥镜检查，没有一个接受阴道GVHD检查。原发性卵巢功能不全（POI）的检查结果可能与GVHD的检查结果重叠，并且我们队列中的6例患者（32％）被诊断为POI。在诊断外阴阴道GVHD时，只有1例患者正在接受全身性激素替代治疗。大多数患者（n = 16）接受局部类固醇治疗，中位反应时间为43天。5名患者（26％）对治疗有完全缓解，10名患者（53％）有部分缓解。