Sweet's Syndrome Complicated by Kikuchi's Disease and Hemophagocytic Syndrome which Presented with Retinoic Acid-inducible Gene-I in both the Skin Epidermal Basal Layer and the Cervical Lymph Nodes

Tomohiro Koga; Kanako Takano; Yoshiro Horai; Satoshi Yamasaki; Hideki Nakamura; Akinari Mizokami; Koichi Ohshima; Atsushi Kawakami

doi:10.2169/internalmedicine.52.9542

CASE REPORTS

Sweet's Syndrome Complicated by Kikuchi's Disease and Hemophagocytic Syndrome which Presented with Retinoic Acid-inducible Gene-I in both the Skin Epidermal Basal Layer and the Cervical Lymph Nodes

Tomohiro Koga, Kanako Takano, Yoshiro Horai, Satoshi Yamasaki, Hideki Nakamura, Akinari Mizokami, Koichi Ohshima, Atsushi Kawakami

著者情報

Tomohiro Koga
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Kanako Takano
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Yoshiro Horai
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Satoshi Yamasaki
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Hideki Nakamura
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan
Akinari Mizokami
Department of Rheumatology, Nagasaki Municipal Hospital, Japan
Koichi Ohshima
Department of Pathology, Kurume University School of Medicine, Japan
Atsushi Kawakami
Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan

キーワード: hemophagocytic syndrome, Kikuchi's disease, retinoic acid-inducible gene-I, Sweet's syndrome

ジャーナルオープンアクセス

2013 年 52 巻 16 号 p. 1839-1843

DOI https://doi.org/10.2169/internalmedicine.52.9542

詳細

抄録

A 21-year-old man was admitted to our hospital with a fever, erythema, cervical lymphadenopathy and pancytopenia. A diagnosis of Sweet's syndrome (SS) with Kikuchi's disease (KD) and hemophagocytic syndrome (HPS) was made based on the results of a bone marrow aspiration along with the results from biopsy specimens of the brachial skin and a cervical lymph node. The expression of retinoic acid-inducible gene-I (RIG-I) in the skin epidermal basal layer as well as in a cervical lymph node was revealed through immunohistochemistry. He successfully entered remission through treatment with prednisolone. This findings of this case indicate that when SS with KD presents as HPS, it may suggest an association with an RIG-I-related innate immunity.

責任著者(Corresponding author)

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