朗格汉斯组织细胞增生症引起的甲损害

Nail changes in Langerhans cell histiocytosis

朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的皮肤损害较多见,而甲损害则罕见。其临床上可表现为指、趾甲的甲周红斑及肿胀、甲襞破坏、纵向开槽、紫癜样纹、甲下紫癜、甲角化过度、甲下脓疱、甲变形、甲板缺失、甲沟炎、甲剥离、纵形红甲和甲蚀斑。报告1例34岁男性患者,具有尿崩症,突眼,骨损害及皮肤黏膜病变,如:头面部脂溢性皮炎样损害,浸润结节和溃疡斑块,特别是在腋下,外生殖器区域。指甲损害表现为甲周红斑及肿胀、甲分离、甲下紫癜、裂片样出血、纵向开槽及纵形红甲。皮损组织病理检查证实为LCH。成人皮肤弥漫性LCH罕见,误诊率高,确诊应结合组织病理学特征、免疫表型及超微结构检查综合分析,甲损害可能是该病系统病变的征象,但其是否为不良预后的标志却未可知。

Skin lesions occur frequently in Langerhans cell histiocytosis （LCH） whereas nail lesions are rare. Nail lesion, is characterized by various features like paronychial erythema, swelling nail fold destruction, longitudinal grooving, purpuric striae, subungual purpura hyperkeratosis, subungual pustules, deformity, loss of nail plate, paronychia, onycholysis, longitudinal ery- thronychia splinter hemorrhages and pitting of the fingernails and toenails. A 34-year-old male presented with diabetes in- sipidus, exophthalmos and bone defects and mucoeutaneous lesions, including seborrheic dermatitis on the face and scalp, infiltrated nodules and ulcerated plaques, especially in the axillae and anogenital region. Nail changes included paronychial erythema, swelling nail fold destruction, subungual purpura, onycholysis, splinter hemorrhages, longitudinal grooving and longitudi nal erythronychia. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of skin biopsies. LCH is rare and easy to be misdiagnosed. The confirmed diagnosis is based on the aggregate analysis of its histological features,nail changes in Langerhans cell histiocytosis may be a possible marker of muhisystem disease. The role of nail lesion as an unfavorable prognostic sign is unclear.