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. 2023 Sep-Oct;98(5):651-655.
doi: 10.1016/j.abd.2022.07.010. Epub 2023 Jun 8.

The evaluation of IgG4 and IgG expression in cutaneous Rosai-Dorfman disease

Affiliations

The evaluation of IgG4 and IgG expression in cutaneous Rosai-Dorfman disease

Puyu Zou et al. An Bras Dermatol. 2023 Sep-Oct.

Abstract

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.

Keywords: Histiocytosis; Immunoglobulin G; Immunoglobulin G4-related disease; Skin.

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Figures

Figure 1
Figure 1
(A) Infiltrating plaque type: infiltrating yellow-red plaque, with uneven surface, and multiple dark red nodules of different sizes at the edge. (B) Nodular papule type: local distribution of multiple red papules of 0.2‒0.5 cm in size on the surface of pale erythema, showing isolated and non-fused shape
Figure 2
Figure 2
Tumor like type: Dark red palm-sized mass on flexor aspect of left thigh with obvious central uplift and multiple dark red nodules in the surrounding surface
Figure 3
Figure 3
(A) It showed a diffused infiltration of large histiocytes, lymphocytes, and plasma cells with scattered neutrophils. (B) Expression of IgG. (C) Expression of IgG4

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