Mansfield Patricia K, Tinker Daniel, Smith Emily, Burkemper Nicole M, Smith Kristin, Guo Aibing Mary
Saint Louis University School of Medicine, St. Louis, MO.
J Low Genit Tract Dis. 2025 Apr 1;29(2):195-200. doi: 10.1097/LGT.0000000000000871. Epub 2025 Jan 22.
Authors characterized all published adult cases of cutaneous, intertriginous Langerhans cell histiocytosis (LCH) to bring this clinical presentation to the attention of clinicians. The authors emphasize the morphology, histopathology, immunohistochemical profiles, and genetic mutations associated with these cases.
A systematic review of the National Center for Biotechnology Information's PubMed was conducted, utilizing the following specific key words to identify all adult LCH patients with cutaneous intertriginous involvement: "Intertriginous Langerhans," "Vulvar Langerhans," "Genital Langerhans," "Perineal Langerhans," "Perianal Langerhans," "Intergluteal Langerhans," "Inguinal Langerhans," "Axillary Langerhans," and "Inframammary Langerhans." Reports were subjected to strict inclusion criteria: case reports, case series, or meta-analyses documenting case(s) of biopsy-proven LCH with cutaneous, intertriginous involvement in adult patients (>18 years of age at the time of diagnosis).
This systematic review identified 1 original and 121 published cases of biopsy-proven, cutaneous, intertriginous LCH in adult patients. Morphology commonly included eroded, ulcerated papules and plaques, and rare presentations demonstrated potential mimickers (hidradenitis suppurativa, deep fungal mycosis, condyloma accuminata).
This systematic review encompasses the largest compilation of adult cutaneous intertriginous LCH cases in the medical literature to the authors' knowledge to date. This study identifies an important clinical presentation of this rare, commonly pediatric diagnosis; highlights trends among these cases and important clinical mimickers; and serves as a reminder to clinicians to maintain suspicion for LCH in adult populations, particularly in the setting of intertriginous cutaneous involvement.
作者对所有已发表的成人皮肤、间擦部位朗格汉斯细胞组织细胞增生症（LCH）病例进行特征分析，以引起临床医生对这种临床表现的关注。作者强调了与这些病例相关的形态学、组织病理学、免疫组化特征及基因突变情况。
对美国国立生物技术信息中心的PubMed数据库进行系统综述，使用以下特定关键词来识别所有有皮肤间擦部位受累的成人LCH患者：“间擦部位朗格汉斯”“外阴朗格汉斯”“生殖器朗格汉斯”“会阴朗格汉斯”“肛周朗格汉斯”“臀间沟朗格汉斯”“腹股沟朗格汉斯”“腋窝朗格汉斯”和“乳房下朗格汉斯”。纳入的报告需符合严格的标准：病例报告、病例系列或荟萃分析，记录经活检证实的成人患者（诊断时年龄>18岁）皮肤间擦部位受累的LCH病例。
该系统综述共识别出1篇原创病例及121篇已发表的经活检证实的成人皮肤间擦部位LCH病例。形态学表现通常包括糜烂、溃疡的丘疹和斑块，少数病例表现出可能的模仿疾病（化脓性汗腺炎、深部真菌病、尖锐湿疣）。
据作者所知，该系统综述涵盖了医学文献中最大规模的成人皮肤间擦部位LCH病例汇编。本研究确定了这种罕见的、通常在儿童期诊断的疾病的一种重要临床表现；突出了这些病例中的趋势以及重要的临床模仿疾病；并提醒临床医生在成人人群中，尤其是在皮肤间擦部位受累的情况下，要对LCH保持警惕。
