【摘要】 报告1例发热性溃疡坏死性急性痘疮样糠疹。患儿男,8岁。全身红斑、水疱、坏死20 d,伴有持续高热、末梢血白细胞和丙氨酸转氨酶显著升高、低蛋白血症等,病情危重。应用大剂量糖皮质激素治疗的同时,加用大剂量静脉注射用人免疫球蛋白冲击治疗取得较好效果。更多还原

【Abstract】 Febrile ulceronecrotic Mucha-Habermann’s disease (FUMHD) is an unusual severe form of pityriasis lichenoides et varioliformis acuta..To our knowledge,only 19 cases of this disease have been reported in the literature,and 4 of them were dead.We report a case of this disorder.An 8-year-old boy presented with persistently high fever,hypoproteinemia,significantly high peripheral blood leukocytes count and elevated liver transaminase level.He was successfully treated with methylprednisolone and subsequently high doses of intravenous immunoglobulin (IVIG).According to the literature,the treatment of FUMHD remains primarily methotrexate and high-dose of corticosteroids.No report of administration of IVIG for FUMHD in the literature till now,the good response of this patient indicates it could be used in cases refractory to other treatments.更多还原