Department of Pathology, Hospital Universitario 12 de Octubre, Universidad, Complutense, Instituto de Investigación I+12, Madrid, Spain.
Department of Dermatology, Reina Sofía University Hospital, Córdoba, Spain; and.
Am J Dermatopathol. 2020 May;42(5):356-359. doi: 10.1097/DAD.0000000000001572.
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder, more frequent in children, characterized by an abnormal accumulation of Langerhans cells admixed with eosinophils, lymphocytes, neutrophils, and macrophages. The clinical presentation is variable and depends on whether a single or multiple organs are affected. Skin lesions are common in LCH (40% of cases) and represent a frequent form of presentation (in up to 80% of cases). Cutaneous manifestations of LCH are highly variable, frequently presenting as crusted papules or scaly seborrheic-like lesions localized in the scalp. We report the first case of a localized acral sclerosing LCH, a new form of LCH. This case highlights the broad and surprising form of presentation of LCH which may be overlooked and can significantly delay its diagnosis. The development of systemic disease may occur months to years after the initial skin presentation. Prompt diagnosis and treatment may prevent progression to systemic disease, as documented in some cases.
朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的增生性疾病，多见于儿童，其特征是异常积聚朗格汉斯细胞，伴嗜酸性粒细胞、淋巴细胞、中性粒细胞和巨噬细胞。临床表现多种多样，取决于受累的是单个器官还是多个器官。皮肤病变在 LCH 中很常见（占 40%），是一种常见的表现形式（高达 80%的病例）。LCH 的皮肤表现变化多样，常表现为结痂丘疹或鳞屑性脂溢样病变，局限于头皮。我们报告首例局限性肢端硬化性 LCH，这是 LCH 的一种新形式。该病例突出了 LCH 广泛而令人惊讶的表现形式，可能会被忽视，并可能显著延迟其诊断。全身性疾病可能在最初的皮肤表现后数月至数年内发生。一些病例证实，及时诊断和治疗可能防止疾病进展为全身性疾病。
