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

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

Intern Med. 2013;52(16):1839-43. doi: 10.2169/internalmedicine.52.9542. Epub 2012 Mar 1.

Authors

Tomohiro Koga¹, Kanako Takano, Yoshiro Horai, Satoshi Yamasaki, Hideki Nakamura, Akinari Mizokami, Koichi Ohshima, Atsushi Kawakami

Affiliation

¹ Unit of Translational Medicine, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Japan. [email protected]

PMID: 23955621
DOI: 10.2169/internalmedicine.52.9542

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. 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.

Publication types

Case Reports

MeSH terms

DEAD Box Protein 58
DEAD-box RNA Helicases / biosynthesis
DEAD-box RNA Helicases / metabolism*
Epidermis / metabolism*
Epidermis / pathology
Histiocytic Necrotizing Lymphadenitis / complications
Histiocytic Necrotizing Lymphadenitis / diagnosis
Histiocytic Necrotizing Lymphadenitis / metabolism*
Humans
Immunity, Innate
Lymph Nodes / metabolism*
Lymph Nodes / pathology
Lymphohistiocytosis, Hemophagocytic / complications
Lymphohistiocytosis, Hemophagocytic / diagnosis
Lymphohistiocytosis, Hemophagocytic / metabolism*
Male
Neck
Receptors, Immunologic
Sweet Syndrome / complications
Sweet Syndrome / diagnosis
Sweet Syndrome / metabolism*
Young Adult

Substances

Receptors, Immunologic
RIGI protein, human
DEAD Box Protein 58
DEAD-box RNA Helicases