Mataix Javier, Betlloch Isabel, Lucas-Costa Ana, Pérez-Crespo María, Moscardó-Guilleme Cristina
Department of Dermatology, Hospital General Universitario de Alicante, Alicante, Spain.
Pediatr Dermatol. 2008 Mar-Apr;25(2):247-51. doi: 10.1111/j.1525-1470.2008.00645.x.
We describe a child with a 6-month history of onycholysis, subungual hyperkeratosis, and hemorrhages in most of her fingernails and toenails. Nail involvement preceded the identification of osteolytic lesions at the mastoid on a cranial computed tomography scan, which was performed because of repeated episodes of acute otitis media. Some weeks later, a small number of erythematous papules developed over the trunk and face. The diagnosis of Langerhans cell histiocytosis was made by histopathologic examination of bone, skin, and bed and matrix nail biopsies. Response to treatment with vinblastine and prednisone was excellent. Nail changes in Langerhans cell histiocytosis are extremely uncommon, particularly as the presenting manifestation of the disease. The role of nail involvement as an unfavorable prognostic sign is unclear.
我们描述了一名儿童，其大多数手指甲和脚趾甲出现甲剥离、甲下角化过度及出血已有6个月病史。在因反复急性中耳炎发作而进行的头颅计算机断层扫描中，发现乳突有溶骨性病变，指甲受累早于此。几周后，躯干和面部出现少量红斑丘疹。通过对骨骼、皮肤、甲床及甲母质活检进行组织病理学检查，诊断为朗格汉斯细胞组织细胞增多症。长春碱和泼尼松治疗反应良好。朗格汉斯细胞组织细胞增多症中的指甲改变极为罕见，尤其是作为该疾病的首发表现。指甲受累作为不良预后征象的作用尚不清楚。
