Open AccessTissue-infiltrating immune cells contribute to understanding the pathogenesis of Kimura disease
Author(s) -
Takashi Mitsui,
Ryusuke Munemura,
Masakata Shimizu,
Noriko Kakizoe,
Naoki Kaneko,
Yasuki Murakami,
Masafumi Moriyama,
Tamotsu Kiyoshima,
Shintaro Kawano,
Seiji Nakamura
Publication year - 2019
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000018300
Subject(s) - medicine , immunoglobulin e , eosinophil , gata3 , eosinophilia , pathogenesis , antibody , immune system , kimura's disease , pathology , immunology , asthma , biology , biochemistry , transcription factor , gene
Rationale: Kimura disease (KD) is a rare, chronic inflammatory disorder characterized by subcutaneous granuloma in the head and neck region, as well as increased eosinophil counts and high serum immunoglobulin E (IgE) levels. Kimura disease is suspected to be an IgE-mediated disease, associated with an allergic response, in which antigen-specific B cells are stimulated to undergo specific IgE class switching with disease-specific CD4+ T (Th) cells help. Thus, exploration of the Th cells in affected tissues with KD is a highly promising field of the investigation. However, there have been no reports with direct evidence to implicate Th cells in affected lesions with KD. Here we quantitatively demonstrate that CD4+ GATA3+ T cells and interleukin (IL)-4+ IgE+ c-kit+ mast cells prominently infiltrate in affected lesion with KD. Patient concerns: A 56-year-old Japanese man who exhibited painless swelling in the left parotid region. Diagnoses: Diagnosis of KD was made based on characteristic histopathologic findings, in conjunction with peripheral eosinophilia and elevated serum IgE levels. Interventions: The patient underwent corticosteroid therapy and had been followed for 2 years. Outcomes: We report a rare case of KD of the parotid region in a 56-year-old man, followed by corticosteroid therapy for 2 years. The mass decreased in size and skin itchiness decreased after therapy. He was discharged without any complications. Furthermore, we quantitatively demonstrate the dominance of CD4+ GATA3+ T cells in affected tissues of KD and detect IL-4+ IgE+ c-kit+ mast cells in lesions by multicolor staining approaches. Lessons: The findings from this case suggest that peripheral blood eosinophilia might serve as a marker of recurrent disease, long-term follow-up is necessary due to the possibility of recurrent. Interactions among expanded IgE+ B cells, CD4+ GATA3+ T cells, eosinophils, and activated mast cells might play a critical role in the pathogenesis of KD.