Tissue-infiltrating immune cells contribute to understanding the pathogenesis of Kimura disease: A case report.

Abstract

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.

• Authors: Takashi Maehara, Ryusuke Munemura, Mayumi Shimizu, Noriko Kakizoe, Naoki Kaneko, Yuka Murakami, Moriyama Masafumi, Tamotsu Kiyoshima, Shintaro Kawano, Seiji Nakamura
• Journal: Medicine (Medicine (Baltimore)) Vol. 98 Issue 50 Pg. e18300 (Dec 2019) ISSN: 1536-5964 [Electronic] United States
• PMID: 31852109 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin E

Topics

• Angiolymphoid Hyperplasia with Eosinophilia (blood, diagnosis, immunology)
• B-Lymphocytes (immunology, pathology)
• Biopsy
• Eosinophils (immunology, pathology)
• Humans
• Immunoglobulin E (blood)
• Magnetic Resonance Imaging
• Male
• Mast Cells (immunology, pathology)
• Middle Aged