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The evaluation of IgG4 and IgG expression in cutaneous Rosai-Dorfman disease
Puyu Zou1, Yi Zhan1, Ruzeng Xue2
1Department of Dermatology, Second Xiangya Hospital, Central South University, Changsha, China.
Insights
Immunoglobulin G4 (IgG4) and IgG expression in cutaneous Rosai-Dorfman Disease (CRDD) were investigated. Elevated IgG4 and IgG levels, along with their ratio, may offer insights into CRDD pathogenesis.
Area of Science:
- Immunopathology
- Dermatology
- Histopathology
Background:
- Cutaneous Rosai-Dorfman Disease (CRDD) is a rare benign histiocytic disorder.
- The exact pathogenesis of CRDD remains incompletely understood.
- Investigating immunoglobulin expression may elucidate disease mechanisms.
Purpose of the Study:
- To investigate the expression of Immunoglobulin G4 (IgG4) and Immunoglobulin G (IgG) in CRDD.
- To enhance the understanding of CRDD pathogenesis through immunoglobulin analysis.
Main Methods:
- Retrospective analysis of 23 CRDD patient cases.
- Diagnosis confirmed by emperipolesis and specific immunohistochemical (IHC) markers (S-100, CD68, CD1a).
- Quantitative assessment of IgG and IgG4 expression in cutaneous specimens using IHC and image analysis.
Main Results:
- CRDD confirmed in 23 patients (14 males, 9 females; age range 17-68).
- Commonly affected sites included the face, trunk, ears, and neck.
- IgG positivity was observed in 22 cases, and IgG4 positivity in 18 cases, with a variable IgG4/IgG ratio.
Conclusions:
- The positive rates of IgG4 and IgG, and their ratio, are significant findings.
- These immunoglobulin markers may play a role in understanding CRDD pathogenesis.
- Further multi-center studies are warranted due to the small sample size.
Objective:
The authors investigated the expression of IgG4 and IgG in cutaneous Rosai-Dorfman Disease (CRDD) to further improve the understanding of this disease.
Methods:
The authors retrospectively reviewed the clinicopathological features of 23 CRDD patients. The authors diagnosed CRDD by the presence of emperipolesis and immunohistochemical (IHC) staining of histiocytes consisting of S-100(+)/CD68(+)/CD1a(-) cells. The expressions of IgG and IgG4 in cutaneous specimens were assessed by IHC (EnVision) and quantitatively calculated by a medical image analysis system.
Results:
All 23 patients, including 14 males and 9 females, were confirmed to have CRDD. Their ages ranged from 17 to 68 years (mean 47.91 ± 14.16). The most frequently affected skin regions were the face, followed by the trunk, ears, neck, limbs, and genitals. In 16 of these cases, the disease presented as a single lesion. IHC staining of sections showed that IgG was positive (≥ 10 cells/High-Power Field [HPF]) in 22 cases, while IgG4 was positive (≥ 10 cells/HPF) in 18 cases. Moreover, the IgG4/IgG proportion ranged from 1.7% to 85.7% (mean 29.50 ± 24.67%, median 18.4%) in the 18 cases.
Study Limitations:
In the majority of studies, as well as in the current study, the design. RDD is a rare disease, so the sample size is small. In the next studies to come, the authors will expand the sample for multi-center verification and in-depth study.
Conclusion:
The positive rates of IgG4 and IgG and the IgG4/IgG ratio assessed through IHC staining may be important in understanding the pathogenesis of CRDD.
