Related Experiment Video
Updated: Mar 24, 2026
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Cutaneous manifestations of IgG4-related disease (RD): A systematic review
Alexandra Charrow1, Sotonye Imadojemu2, Sasha Stephen3
1Departments of Internal Medicine and Dermatology, Harvard University, Boston, Massachusetts.
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.
Related Concept Videos
01:30Inflammatory Bowel Disease II: Crohn's Disease
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
01:19Hypersensitivity Reactions: Immune-Complex Reactions
01:30Hypersensitivities
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
01:17Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:27Inflammatory Bowel Disease I: Ulcerative Colitis
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
01:23Skin Diseases and Disorders
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...