Gao Xiaofei, Yu Bin, Huang Jiaotian, Yang Haixia, Wei Zhu
Department of Dermatology, Hunan Children's Hospital, Changsha, China.
Department of Pediatric Intensive Care Unit (PICU), Hunan Children's Hospital, Changsha, China.
Case Rep Dermatol. 2025 Apr 21;17(1):165-173. doi: 10.1159/000545567. eCollection 2025 Jan-Dec.
Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm that can involve nearly any organ, leading to multisystem damage. Nail involvement in LCH is particularly uncommon. Here we report a case of a young boy with multisystem LCH initially presenting with nail changes.
We described a boy who presented with a 2-year history of asymptomatic changes characterized by onycholysis, subungual hyperkeratosis, and purpuric striae affecting most fingernails and toenails, initially attributed to onychomycosis. Two years later, he developed multisystem involvement affecting the pituitary gland, lungs, skin, liver, and spleen. The patient succumbed shortly after histopathological confirmation via skin biopsy due to massive gastrointestinal hemorrhage secondary to cirrhosis-induced portal hypertension.
Nail lesions may serve as the initial manifestation of LCH, often preceding other characteristic disease symptoms. This early presentation provides critical diagnostic opportunities for timely intervention. Consideration of LCH, biopsy, and comprehensive evaluation of organ involvement is essential to reduce the rate of misdiagnosis and the potential for unrecognized high-risk disease.
Nail involvement in LCH, while rare, may serve as an early clinical indicator of multisystem disease.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的髓系肿瘤，可累及几乎任何器官，导致多系统损害。LCH累及指甲尤为罕见。本文报告一例多系统LCH的年轻男孩，最初表现为指甲改变。
我们描述了一名男孩，有2年无症状改变的病史，表现为甲剥离、甲下角化过度和紫癜样条纹，累及多数手指甲和脚趾甲，最初归因于甲癣。两年后，他出现多系统受累，累及垂体、肺、皮肤、肝脏和脾脏。经皮肤活检组织病理学确诊后不久，患者因肝硬化所致门静脉高压继发大量胃肠道出血而死亡。
指甲病变可能是LCH的初始表现，通常早于其他特征性疾病症状。这种早期表现为及时干预提供了关键的诊断机会。考虑LCH、活检以及对器官受累情况进行全面评估对于降低误诊率和未识别的高危疾病风险至关重要。
LCH累及指甲虽罕见，但可能是多系统疾病的早期临床指标。
