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
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 resu...
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| Veröffentlicht in: | Internal Medicine 2013, Vol.52(16), pp.1839-1843 |
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| container_title | Internal Medicine |
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| creator | Koga, Tomohiro Takano, Kanako Horai, Yoshiro Yamasaki, Satoshi Nakamura, Hideki Mizokami, Akinari Ohshima, Koichi Kawakami, Atsushi |
| description | 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. |
| doi_str_mv | 10.2169/internalmedicine.52.9542 |
| format | Article |
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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.</description><identifier>ISSN: 0918-2918</identifier><identifier>EISSN: 1349-7235</identifier><identifier>DOI: 10.2169/internalmedicine.52.9542</identifier><identifier>PMID: 23955621</identifier><language>eng</language><publisher>Japan: The Japanese Society of Internal Medicine</publisher><subject>DEAD Box Protein 58 ; DEAD-box RNA Helicases - biosynthesis ; DEAD-box RNA Helicases - metabolism ; Epidermis - metabolism ; Epidermis - pathology ; hemophagocytic syndrome ; Histiocytic Necrotizing Lymphadenitis - complications ; Histiocytic Necrotizing Lymphadenitis - diagnosis ; Histiocytic Necrotizing Lymphadenitis - metabolism ; Humans ; Immunity, Innate ; Kikuchi's disease ; Lymph Nodes - metabolism ; Lymph Nodes - pathology ; Lymphohistiocytosis, Hemophagocytic - complications ; Lymphohistiocytosis, Hemophagocytic - diagnosis ; Lymphohistiocytosis, Hemophagocytic - metabolism ; Male ; Neck ; Receptors, Immunologic ; retinoic acid-inducible gene-I ; Sweet Syndrome - complications ; Sweet Syndrome - diagnosis ; Sweet Syndrome - metabolism ; Sweet's syndrome ; Young Adult</subject><ispartof>Internal Medicine, 2013, Vol.52(16), pp.1839-1843</ispartof><rights>2013 by The Japanese Society of Internal Medicine</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c604t-dd32285f97eb0aca15b9709645628282e2135477c064fb85c08f8681efd9f5e93</citedby><cites>FETCH-LOGICAL-c604t-dd32285f97eb0aca15b9709645628282e2135477c064fb85c08f8681efd9f5e93</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,781,785,1884,27928,27929</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/23955621$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Koga, Tomohiro</creatorcontrib><creatorcontrib>Takano, Kanako</creatorcontrib><creatorcontrib>Horai, Yoshiro</creatorcontrib><creatorcontrib>Yamasaki, Satoshi</creatorcontrib><creatorcontrib>Nakamura, Hideki</creatorcontrib><creatorcontrib>Mizokami, Akinari</creatorcontrib><creatorcontrib>Ohshima, Koichi</creatorcontrib><creatorcontrib>Kawakami, Atsushi</creatorcontrib><title>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</title><title>Internal Medicine</title><addtitle>Intern. Med.</addtitle><description>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. 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Takano, Kanako ; Horai, Yoshiro ; Yamasaki, Satoshi ; Nakamura, Hideki ; Mizokami, Akinari ; Ohshima, Koichi ; Kawakami, Atsushi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c604t-dd32285f97eb0aca15b9709645628282e2135477c064fb85c08f8681efd9f5e93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>DEAD Box Protein 58</topic><topic>DEAD-box RNA Helicases - biosynthesis</topic><topic>DEAD-box RNA Helicases - metabolism</topic><topic>Epidermis - metabolism</topic><topic>Epidermis - pathology</topic><topic>hemophagocytic syndrome</topic><topic>Histiocytic Necrotizing Lymphadenitis - complications</topic><topic>Histiocytic Necrotizing Lymphadenitis - diagnosis</topic><topic>Histiocytic Necrotizing Lymphadenitis - metabolism</topic><topic>Humans</topic><topic>Immunity, Innate</topic><topic>Kikuchi's disease</topic><topic>Lymph Nodes - metabolism</topic><topic>Lymph Nodes - pathology</topic><topic>Lymphohistiocytosis, Hemophagocytic - complications</topic><topic>Lymphohistiocytosis, Hemophagocytic - diagnosis</topic><topic>Lymphohistiocytosis, Hemophagocytic - metabolism</topic><topic>Male</topic><topic>Neck</topic><topic>Receptors, Immunologic</topic><topic>retinoic acid-inducible gene-I</topic><topic>Sweet Syndrome - complications</topic><topic>Sweet Syndrome - diagnosis</topic><topic>Sweet Syndrome - metabolism</topic><topic>Sweet's syndrome</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Koga, Tomohiro</creatorcontrib><creatorcontrib>Takano, Kanako</creatorcontrib><creatorcontrib>Horai, Yoshiro</creatorcontrib><creatorcontrib>Yamasaki, Satoshi</creatorcontrib><creatorcontrib>Nakamura, Hideki</creatorcontrib><creatorcontrib>Mizokami, Akinari</creatorcontrib><creatorcontrib>Ohshima, Koichi</creatorcontrib><creatorcontrib>Kawakami, Atsushi</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Koga, Tomohiro</au><au>Takano, Kanako</au><au>Horai, Yoshiro</au><au>Yamasaki, Satoshi</au><au>Nakamura, Hideki</au><au>Mizokami, Akinari</au><au>Ohshima, Koichi</au><au>Kawakami, Atsushi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>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</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. Med.</addtitle><date>2013-01-01</date><risdate>2013</risdate><volume>52</volume><issue>16</issue><spage>1839</spage><epage>1843</epage><pages>1839-1843</pages><issn>0918-2918</issn><eissn>1349-7235</eissn><abstract>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. 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| subjects | DEAD Box Protein 58 DEAD-box RNA Helicases - biosynthesis DEAD-box RNA Helicases - metabolism Epidermis - metabolism Epidermis - pathology hemophagocytic syndrome Histiocytic Necrotizing Lymphadenitis - complications Histiocytic Necrotizing Lymphadenitis - diagnosis Histiocytic Necrotizing Lymphadenitis - metabolism Humans Immunity, Innate Kikuchi's disease Lymph Nodes - metabolism Lymph Nodes - pathology Lymphohistiocytosis, Hemophagocytic - complications Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - metabolism Male Neck Receptors, Immunologic retinoic acid-inducible gene-I Sweet Syndrome - complications Sweet Syndrome - diagnosis Sweet Syndrome - metabolism Sweet's syndrome Young Adult |
| title | 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 |
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