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Immunoglobulin G4-related disease and its skin manifestations.

Abstract Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition characterized by tissue infiltration with IgG4(+) plasma cells and an elevated serum IgG4 level. It usually occurs in middle-aged or older patients, with male predominance, and can involve multiple organs synchronously or metachronously. Patients with IgG4-RD generally respond to steroid therapy. IgG4-related skin lesions are typically erythematous and itchy plaques, subcutaneous nodules, or papules like prurigo nodularis located on the head and neck areas. Histologically, lymphoplasmacytic infiltrates with abundant IgG4+ plasma cells are seen in the dermis and/or subcutaneous tissue. The skin condition of IgG4-RD does not necessarily exhibit infiltration of IgG4(+) plasma cells, which is a prerequisite for IgG4-related skin disease, but such skin lesions without infiltration by IgG4(+) plasma cells can be included in the skin manifestations of IgG4-RD.
PMID
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Authors

Mayor MeshTerms
Keywords

hypergammaglobulinemia

immunoglobulin G4-related disease

immunoglobulin G4-related skin disease

leukocytoclastic vasculitis

skin

Journal Title the journal of dermatology
Publication Year Start




PMID- 28256769
OWN - NLM
STAT- MEDLINE
DA  - 20170303
DCOM- 20170306
LR  - 20170306
IS  - 1346-8138 (Electronic)
IS  - 0385-2407 (Linking)
VI  - 44
IP  - 3
DP  - 2017 Mar
TI  - Immunoglobulin G4-related disease and its skin manifestations.
PG  - 288-296
LID - 10.1111/1346-8138.13723 [doi]
AB  - Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition 
      characterized by tissue infiltration with IgG4+ plasma cells and an elevated
      serum IgG4 level. It usually occurs in middle-aged or older patients, with male
      predominance, and can involve multiple organs synchronously or metachronously.
      Patients with IgG4-RD generally respond to steroid therapy. IgG4-related skin
      lesions are typically erythematous and itchy plaques, subcutaneous nodules, or
      papules like prurigo nodularis located on the head and neck areas.
      Histologically, lymphoplasmacytic infiltrates with abundant IgG4+ plasma cells
      are seen in the dermis and/or subcutaneous tissue. The skin condition of IgG4-RD 
      does not necessarily exhibit infiltration of IgG4+ plasma cells, which is a
      prerequisite for IgG4-related skin disease, but such skin lesions without
      infiltration by IgG4+ plasma cells can be included in the skin manifestations of 
      IgG4-RD.
CI  - (c) 2017 Japanese Dermatological Association.
FAU - Takayama, Ryoko
AU  - Takayama R
AD  - Department of Dermatology, Graduate School of Medicine, Nippon Medical School,
      Tokyo, Japan.
FAU - Ueno, Takashi
AU  - Ueno T
AD  - Department of Dermatology, Graduate School of Medicine, Nippon Medical School,
      Tokyo, Japan.
FAU - Saeki, Hidehisa
AU  - Saeki H
AD  - Department of Dermatology, Graduate School of Medicine, Nippon Medical School,
      Tokyo, Japan.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - England
TA  - J Dermatol
JT  - The Journal of dermatology
JID - 7600545
RN  - 0 (Immunoglobulin G)
SB  - IM
MH  - Humans
MH  - Hypergammaglobulinemia/complications/*immunology
MH  - Immunoglobulin G/*immunology
MH  - Skin Diseases/*diagnosis/etiology/immunology
OTO - NOTNLM
OT  - hypergammaglobulinemia
OT  - immunoglobulin G4-related disease
OT  - immunoglobulin G4-related skin disease
OT  - leukocytoclastic vasculitis
OT  - skin
EDAT- 2017/03/04 06:00
MHDA- 2017/03/07 06:00
CRDT- 2017/03/04 06:00
PHST- 2016/11/04 [received]
PHST- 2016/11/04 [accepted]
AID - 10.1111/1346-8138.13723 [doi]
PST - ppublish
SO  - J Dermatol. 2017 Mar;44(3):288-296. doi: 10.1111/1346-8138.13723.

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