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Investigations of IgG4-related disease involving the skin

  • Original Article
  • Published:
Modern Rheumatology

Abstract

Objectives

IgG4-related skin disease is not widely recognized. This prompted us to investigate the clinical and pathological features of five patients we encountered with IgG4-related disease (IgG4-RD) affecting the skin.

Methods

We investigated the clinical and pathological features of these five patients, including the distribution, onset, and morphology of eruptions, their pathological and immunohistochemical characteristics, and the occurrence of disease in other organs.

Results

The skin lesions were typically erythematous nodules and papules and brown papules like prurigo nodularis, which developed on the face or in the head and neck areas in four patients. Skin lesions were the first clinical manifestation in three patients. All five patients had sialadenitis and/or dacryoadenitis. The mean serum IgG4 concentration was 665.6 ± 410.0 mg/dl. Infiltrations of IgG4-positive plasma cells were observed in both the dermis and subcutaneous tissue. Germinal center formations were seen in three patients. Mild to moderate fibrosis was observed in all patients, and focal obliterative phlebitis in one. The average count of IgG4-positive cells was 67.3/high-power field (23.0–128.6). Wide variation in the numbers of infiltrating IgG4-positive cells was noted.

Conclusion

IgG4-RD appears to have a distinctive clinicopathological presentation in the skin, differentiating it from other cutaneous disorders.

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Acknowledgments

We thank Dr. John H. Stone for his critical reading and discussions of the manuscript, Dr. N. Kurose for his help in preparing the specimens of patient 3, and Mr. John Gelblum for his critical reading of the manuscript. This work was partially supported by the Research Program of Intractable Diseases of the Ministry of Health, Labor, and Welfare of Japan.

Conflict of interest

None.

Author information

Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8641, Japan

    Kazunori Yamada, Yasushi Kakuchi & Mitsuhiro Kawano

  2. Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan

    Yasuhito Hamaguchi & Kazuhiko Takehara

  3. Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan

    Takako Saeki

  4. Division of Endocrinology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Kunimasa Yagi & Naoko Ito

  5. Division of Cardiology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Masakazu Yamagishi

  6. Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Yasuni Nakanuma

Authors
  1. Kazunori Yamada
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  2. Yasuhito Hamaguchi
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  3. Takako Saeki
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  4. Kunimasa Yagi
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  5. Naoko Ito
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  6. Yasushi Kakuchi
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  7. Masakazu Yamagishi
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  8. Kazuhiko Takehara
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  9. Yasuni Nakanuma
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  10. Mitsuhiro Kawano
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Corresponding author

Correspondence to Mitsuhiro Kawano.

Additional information

K. Yamada and Y. Hamaguchi contributed equally.

Electronic supplementary material

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10165_2012_786_MOESM1_ESM.tif

Figure S1. Immunostainings for CD138 and IgG4 of the skin lesions of two patients with non-IgG4-related prurigo nodularis. a, b Patient 1. c, d Patient 2. a, c CD138 staining. b, d IgG4 staining. a–d ×100 (TIFF 630 kb)

10165_2012_786_MOESM2_ESM.tif

Figure S2. Immunostainings for CD138 and IgG4 of the skin lesions of three patients with pseudolymphoma. a, b Patient 1. c, d Patient 2. e, f Patient 3. a, c, e CD138 staining. b, d, f IgG4 staining. IgG4-positive cell infiltrates were not seen in these patients. a–f ×100 (TIFF 1751 kb)

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Yamada, K., Hamaguchi, Y., Saeki, T. et al. Investigations of IgG4-related disease involving the skin. Mod Rheumatol 23, 986–993 (2013). https://doi.org/10.1007/s10165-012-0786-7

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  • DOI: https://doi.org/10.1007/s10165-012-0786-7

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