Department of Biomedical Sciences and Human Oncology, Section of Dermatology, University of Turin, Turin, Italy.
Dermatol Ther. 2010 Jul-Aug;23(4):419-22. doi: 10.1111/j.1529-8019.2010.01343.x.
Febrile ulcero-necrotic Mucha-Habermann disease (FUMHD) is a rare subtype of pityriasis lichenoides et varioliformis acuta (only 41 cases described to date), characterized by an acute onset of ulcero-necrotic papules accompanied by high fever and severe constitutional symptoms. We report a case of a 23-year-old man with a steroid-resistant FUMHD treated by intravenous immunoglobulins (IVIG) combined with methotrexate. Only one case of FUMHD treated by IVIG has been reported to date in literature. Also in our case, IVIG proved to be effective in inducing a dramatic improvement of ulceration and in arresting the appearance of new lesions. Moreover, in our experience we decided to perform a maintenance treatment with extracorporeal photochemotherapy (ECP), to the best of our knowledge not previously used in the treatment of pityriasis lichenoides et varioliformis acuta. ECP, which involves extracorporeal exposure of peripheral blood mononuclear cells to photo-activated 8-methoxypsoralen, induces an immunological reaction against auto-reactive T cell clones, without immune-depression and thus could potentially be useful particularly in FUMHD avoiding the risk of an infective reactivation.
发热性溃疡性坏死性蕈样肉芽肿病（FUMHD）是急性痘疮样苔藓样糠疹（急性痘疮样苔藓样糠疹）的一种罕见亚型（迄今为止仅描述了 41 例），其特征为伴有高热和严重全身症状的溃疡性坏死性丘疹急性发作。我们报告了一例 23 岁男性类固醇难治性 FUMHD 患者，采用静脉注射免疫球蛋白（IVIG）联合甲氨蝶呤治疗。迄今为止，文献中仅报道了一例 FUMHD 采用 IVIG 治疗的病例。同样在我们的病例中，IVIG 被证明在诱导溃疡显著改善和阻止新病变出现方面非常有效。此外，根据我们的经验，我们决定采用体外光化学疗法（ECP）进行维持治疗，据我们所知，ECP 以前从未用于治疗急性痘疮样苔藓样糠疹。ECP 涉及外周血单个核细胞在体外暴露于光激活的 8-甲氧基补骨脂素，引发针对自身反应性 T 细胞克隆的免疫反应，而不会产生免疫抑制，因此在 FUMHD 中可能特别有用，可避免感染再激活的风险。
