Sankilampi Ulla, Huikko-Tarvainen Sari, Kärjä Vesa, Pirinen Elina, Naukkarinen Anita, Hollmén Antero
Department of Pediatrics, Kuopio University Hospital, Kuopio, Finland.
J Pediatr Hematol Oncol. 2008 Mar;30(3):245-8. doi: 10.1097/MPH.0b013e318161a9cb.
Congenital Langerhans cell histiocytosis (LCH) is a rare condition with great diversity. A case of congenital skin-only LCH presenting as a "blueberry muffin baby" with a spontaneous regression by the age of 8 months is reported here. New insights into clinical manifestations and prognosis, which is not uniformly positive, are discussed. A thorough examination and a careful follow-up should be provided to these patients. Systemic therapy is warranted in multi-system disease; no consensus on treatment exists in case of LCH isolated to skin. The diagnosis of congenital self-healing LCH should be made only retrospectively.
先天性朗格汉斯细胞组织细胞增多症（LCH）是一种罕见且具有高度异质性的疾病。本文报告了一例仅累及皮肤的先天性LCH病例，该患儿表现为“蓝莓松饼宝宝”，并在8个月大时自发消退。文中讨论了关于临床表现和预后的新见解，其预后并非一概良好。应对这些患者进行全面检查和仔细随访。多系统疾病需要进行全身治疗；对于仅累及皮肤的LCH病例，治疗尚无共识。先天性自愈性LCH的诊断应仅在回顾性时做出。
