IgG4-related skin disease

Summary IgG4‐related disease (IgG4‐RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4+ plasma cells. IgG4‐RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and l...

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Bibliographic Details
Published in:British journal of dermatology (1951) 2014-11, Vol.171 (5), p.959-967
Main Authors: Tokura, Y., Yagi, H., Yanaguchi, H., Majima, Y., Kasuya, A., Ito, T., Maekawa, M., Hashizume, H.
Format: Article
Language:English
Subjects:
Angiolymphoid Hyperplasia with Eosinophilia - immunology
Autoimmune Diseases - classification
Autoimmune Diseases - immunology
Biological and medical sciences
Cheek
Dermatology
Eosinophilia
Erythema - immunology
Fingers - blood supply
Gangrene
Humans
Hyperplasia
Immunoglobulin G
Immunoglobulin G - immunology
Immunoglobulin G - metabolism
Ischemia - immunology
Lacrimal gland and Nasolacrimal duct
Mandible
Medical sciences
Metastases
Mikulicz' Disease - immunology
Mimicry
Nodules
Pancreas
Plaques
Plasma
Plasma cells
Plasma Cells - immunology
Plasmacytoma - immunology
Plasmacytosis
Pseudolymphoma - immunology
Psoriasis
Psoriasis - immunology
Psoriasis vulgaris
Purpura
Purpura, Hyperglobulinemic - immunology
Skin diseases
Skin Diseases - classification
Skin Diseases - immunology
Skin Diseases, Papulosquamous - immunology
Skin lesions
Tumors
Urticaria - immunology
Vasculitis
Vasculitis - immunology
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Description
Summary:Summary IgG4‐related disease (IgG4‐RD) is a recently established clinical entity characterized by high levels of circulating IgG4, and tissue infiltration of IgG4+ plasma cells. IgG4‐RD exhibits a distinctive fibroinflammatory change involving multiple organs, such as the pancreas and salivary and lacrimal glands. The skin lesions of IgG4‐RD have been poorly characterized and may stem not only from direct infiltration of plasma cells but also from IgG4‐mediated inflammation. Based on the documented cases together with ours, we categorized the skin lesions into seven subtypes: (1) cutaneous plasmacytosis (multiple papulonodules or indurations on the trunk and proximal part of the limbs), (2) pseudolymphoma and angiolymphoid hyperplasia with eosinophilia (plaques and papulonodules mainly on the periauricular, cheek and mandible regions), (3) Mikulicz disease (palpebral swelling, sicca syndrome and exophthalmos), (4) psoriasis‐like eruption (strikingly mimicking psoriasis vulgaris), (5) unspecified maculopapular or erythematous eruptions, (6) hypergammaglobulinaemic purpura (bilateral asymmetrical palpable purpuric lesions on the lower extremities) and urticarial vasculitis (prolonged urticarial lesions occasionally with purpura) and (7) ischaemic digit (Raynaud phenomenon and digital gangrene). It is considered that subtypes 1–3 are induced by direct infiltration of IgG4+ plasma cells, while the other types (4–7) are caused by secondary mechanisms. IgG4‐related skin disease is defined as IgG4+ plasma‐cell‐infiltrating skin lesions that form plaques, nodules or tumours (types 1–3), but may manifest secondary lesions caused by IgG4+ plasma cells and/or IgG4 (types 4–7). What is already known about this topic? IgG4‐related disease (IgG4‐RD) is a recently established clinical entity characterized by fibroinflammatory lesions, high levels of circulating IgG4 and tissue infiltration of IgG4+ plasma cells. What does this study add? We comprehensively categorized the skin lesions of IgG4‐RD into primary lesions with direct infiltration of IgG4+ plasma cells (three subtypes) and secondary nonspecific inflammatory lesions where the role of IgG4 remains to be elucidated (four subtypes). Our study clarifies IgG4‐related skin disease and its differential diagnoses.
ISSN:0007-0963
1365-2133
DOI:10.1111/bjd.13296