In IgG4-related disease (IgG4-RD), lesions are known to develop in organs throughout the body, but reports of disease involving the skin are few. We investigated five cases with IgG4-related skin disease who have recently consulted our hospital. The age at onset of the five cases was 53–78 years, and there were four men and one woman. The rash was characterized clinically as multiple erythematous papules in two and subcutaneous nodules, erythematous papules, and multiple brown papules like prurigo nodularis in one each. The histopathological characteristics included eosinophilic infiltrates in all cases and lymphoid follicle formation in four. The cellular infiltrates were primarily perivascular in nature. Fibrosis, although mild, was found in all cases, but obliterative phlebitis was identified in only a single case. Larger series that describe the cutaneous manifestations of IgG4-RD are required in order to develop a more complete understanding of the dermatological features of this disease.
KeywordsSubcutaneous Adipose Tissue Oral Steroid Eosinophil Infiltration Subcutaneous Nodule Accessory Organ
- 11.Yamada K, Hamaguchi Y, Saeki T, Yagi K, Ito N, Kakuchi Y, Yamagishi M, Takehara K, Nakanuma Y, Kawano M. Investigations of IgG4-related disease involving the skin. Mod Rheumatol. 2013; in pressGoogle Scholar