Igg4-Related Skin Disease

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2021-01-18
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IgG4-related skin disease is the recommended name for skin manifestations in IgG4-related disease (IgG4-RD). Multiple different skin manifestations have been described.

Classification

Although a clear understanding of the various skin lesions in IgG4-related disease is a work in progress, skin lesions have been classified into subtypes based on documented cases:

  • Angiolymphoid hyperplasia with eosinophilia (or lesions that mimic it) and cutaneous pseudolymphoma
  • Cutaneous plasmacytosis
  • Eyelid swelling (as part of Mikulicz's disease)
  • Psoriasis-like eruptions
  • Unspecified maculopapular or erythematous eruptions
  • Hypergammaglobulinemic purpura and urticarial vasculitis
  • Impaired blood supply to fingers or toes, leading to Raynaud's phenomenon or gangrene

In addition, Wells syndrome has also been reported in a case of IgG4-related disease.

Note:

  1. ^ Some do not consider cutaneous plasmacytosis to be a feature of IgG4-related disease for reasons that include: a lack of systemic features, no response to steroid therapy and a different histological pattern.

See also

  • IgG4-related disease