Review
Cutaneous manifestations of IgG4-related disease (RD): A systematic review

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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).

Limitations

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.

Section snippets

Search strategy

A comprehensive systematic search of the literature was performed using the electronic database PubMed from January 1990 to August 2013 for all peer-reviewed publications pertaining to cutaneous manifestations of IgG4-RD. Search criteria were defined to include studies with specific key words in the title, abstract, or body. The search terms used were: “IgG4-related disease,” “IgG4-related sclerosing disease,” “hyper-IgG4 disease,” “IgG4-related systemic disease,” “retroperitoneal fibrosis,”

Results

The electronic search and bibliography screen with applied inclusion criteria retrieved 138 references. In all, 35 articles, consisting of case reports and case series, met inclusion criteria (Fig 1).10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43 Three articles including 16 cases were excluded given insufficient information to apply the diagnostic criteria. The remaining 32 articles comprising 58 cases were

Discussion

As IgG4-RD is a recently described multiorgan, systemic inflammatory disease, characteristic findings of cutaneous involvement in IgG4-RD have not to our knowledge been previously reported. This study reviewed 40 cases of cutaneous IgG4-RD. The primary finding was that the distribution of cutaneous IgG4-RD is significantly more likely to involve the head and neck than other areas of the body. Despite the frequency of the cutaneous presentation of papules, plaques, and nodules among cases, no

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    Drs Charrow and Imadojemu contributed equally and are co-first authors.

    Funding sources: None.

    Disclosure: Dr Takeshita has a grant from Pfizer and received payment for CME work related to psoriasis. Dr Lipoff received a Pfizer Independent Grant for Learning and Change from the American Academy of Dermatology. Drs Charrow, Imadojemu, and Ogunleye have no conflicts of interest to declare.

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