Vol.23 No.5

Original Article

Investigations of IgG4-related disease involving the skin

Authors

Kazunori Yamada1 , Yasuhito Hamaguchi2 , Takako Saeki3 , Kunimasa Yagi4 , Naoko Ito4 , Yasushi Kakuchi1 , Masakazu Yamagishi5 , Kazuhiko Takehara2 , Yasuni Nakanuma6 , Mitsuhiro Kawano1

  • Division of Rheumatology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, 13-1 Takara-machi, Kanazawa Ishikawa, 920-8641, Japan
  • Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
  • Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan
  • Division of Endocrinology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
  • Division of Cardiology, Department of Internal Medicine, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
  • Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan
Received:

24 July 2012

Accepted:

12 October 2012

Published online:

1 November 2012

Full Text

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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.

Key words

IgG4-related disease, IgG4-related skin disease, Dermis, Erythematous nodule, Cutaneous pseudolymphoma