title: Cutaneous manifestations of IgG4-related disease (RD): A systematic review.
abstract: BACKGROUND
  IgG4-related disease (RD) is a recently described fibroinflammatory condition with both cutaneous and systemic manifestations. To our knowledge, the cutaneous manifestations have not been well characterized or systematically investigated to date in the literature.
  
  
  OBJECTIVE
  We sought to describe the cutaneous manifestations of IgG4-RD to guide clinical practice, aid in the diagnosis of IgG4-RD, and contribute to the creation of robust cutaneous diagnostic criteria.
  
  
  METHODS
  A systematic search of peer-reviewed publications pertaining to cutaneous manifestations of IgG4-RD yielded 56 cases from 32 case reports and series. The clinical findings among the diagnostic categories were compared.
  
  
  RESULTS
  Forty cases of IgG4-RD with cutaneous disease were identified. Cutaneous head and neck involvement was significantly associated with a diagnosis of IgG4-RD (P = .02). Macules and bullae were not described in any of the included cases. Among cases of systemic IgG4-RD, cutaneous head and neck involvement was most common and statistically significantly associated with the diagnosis of IgG4-RD (P = .001).
  
  
  CONCLUSIONS
  These findings are limited by reporting and publication bias of particular cases and by small sample size.
  
  
  CONCLUSIONS
  Papules, plaques, and nodules of the head and neck appear to characterize patients with cutaneous IgG4-RD, which nevertheless usually presents with systemic manifestations.
authors.affiliation: Departments of Internal Medicine and Dermatology, Harvard University, Boston, Massachusetts.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Charrow
authors.affiliation: Department of Internal Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Sotonye
authors.initials: S
authors.lastname: Imadojemu
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Sasha
authors.initials: S
authors.lastname: Stephen
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Temitayo
authors.initials: T
authors.lastname: Ogunleye
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Junko
authors.initials: J
authors.lastname: Takeshita
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address: jules.lipoff@uphs.upenn.edu.
authors.forename: Jules B
authors.initials: JB
authors.lastname: Lipoff
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D005355:Fibrosis; D006257:Head; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007249:Inflammation / Q000188:drug therapy / Q000276:immunology; D009333:Neck; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology
chemical_list: D007074:Immunoglobulin G
keywords: IgG4-related disease; IgG4-related sclerosing disease; IgG4-related systemic disease; cutaneous manifestations; diagnosis; fibroinflammatory; hyper-IgG4 disease; lymphoplasmacytic infiltrate