Skin Lesions

  • Yasuhito Hamaguchi
  • Kazunori Yamada
  • Takako Saeki
  • Mitsuhiro Kawano
  • Kazuhiko Takehara


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.


Subcutaneous Adipose Tissue Oral Steroid Eosinophil Infiltration Subcutaneous Nodule Accessory Organ 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Cheuk W, Lee KC, Chong LY, Yuen ST, Chan JK. IgG4-related sclerosing disease: a potential new etiology of cutaneous pseudolymphoma. Am J Surg Pathol. 2009;33:1713–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Sato Y, Kojima M, Takata K, Morito T, Asaoku H, Takeuchi T, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric castleman's disease. Mod Pathol. 2009;22:589–99.PubMedCrossRefGoogle Scholar
  3. 3.
    Khosroshahi A, Carruthers MD, Deshpande V, Leb L, Reed JI, Stone JH. Cutaneous immunoglobulin IgG4-related systemic disease. Am J Med. 2011;124:e7–8.PubMedCrossRefGoogle Scholar
  4. 4.
    Hamaguchi Y, Fujimoto M, Matsushita Y, Kitamura-Sawada S, Kawano M, Takehara K. IgG4-related skin disease, a mimic of angiolymphoid hyperplasia with eosinophilia. Dermatology (Basel, Switzerland). 2011;223:301–5.CrossRefGoogle Scholar
  5. 5.
    Kakuchi Y, Yamada K, Suzuki Y, Ito N, Yagi K, Matsumura M, et al. IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis. Intern Med. 2011;50:1465–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Miyagawa-Hayashino A, Matsumura Y, Kawakami F, Asada H, Tanioka M, Yoshizawa A, et al. High ratio of IgG4-positive plasma cell infiltration in cutaneous plasmacytosis–is this a cutaneous manifestation of IgG4-related disease? Hum Pathol. 2009;40: 1269–77.PubMedCrossRefGoogle Scholar
  7. 7.
    Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol. 2010;34:1812–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease. Mod Pathol. 2007;20:884–94.PubMedCrossRefGoogle Scholar
  9. 9.
    Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23:67–71.PubMedCrossRefGoogle Scholar
  10. 10.
    Strehl JD, Hartmann A, Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64: 237–43.PubMedCrossRefGoogle Scholar
  11. 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

Copyright information

© Springer Japan 2014

Authors and Affiliations

  • Yasuhito Hamaguchi
    • 1
  • Kazunori Yamada
    • 2
  • Takako Saeki
    • 3
  • Mitsuhiro Kawano
    • 2
  • Kazuhiko Takehara
    • 1
  1. 1.Department of DermatologyKanazawa UniversityKanazawaJapan
  2. 2.Division of RheumatologyKanazawa University HospitalKanazawaJapan
  3. 3.Department of Internal MedicineNagaoka Red Cross HospitalNagaokaJapan

Personalised recommendations