An Initial Presentation of Facial Impetigo in IgG4-related Disease: A Case Report

Main Article Content

Mildred Min
Jasdeep Sharma

Keywords

IgG4-related disease, sialadenitis, dacryoadenitis, facial impetigo, case report

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is a rare multi-organ, immune-mediated, fibro-inflammatory condition presenting with tumefactive lesions. Although this condition has characteristic clinical and histopathological features, the phenotypic variability of IgG4-RD provides a diagnostic challenge. Here, we describe a case of facial impetigo presenting as a manifestation of IgG4-RD.


Case Presentation: An 18-year-old female presented to the emergency department with acute facial impetigo in the setting of chronic dacryoadenitis, sialadenitis, parotitis, and lymphadenopathy which had been clinically diagnosed as IgG4-RD. The impetiginized facial rash resolved with oral doxycycline, and the steroid-recalcitrant IgG4-RD symptoms responded to rituximab infusions.


 Conclusions: Due to the rarity of IgG4-RD, secondary manifestations and associations of the disease are continuously emerging. Immune-mediated conditions with cutaneous involvement, such as in IgG4-RD, may prime the skin for the inoculation of bacteria and lead to skin infection(s) such as impetigo. Rituximab may be used for steroid-resistant cases of IgG4-RDs without concurrent corticosteroid therapy.

References

1. Karadeniz H, Vaglio A. IgG4-related disease: a contemporary review. Turk J Med Sci. Nov 3 2020;50(SI-2):1616-1631. doi:10.3906/sag-2006-375

2. Chen LYC, Mattman A, Seidman MA, Carruthers MN. IgG4-related disease: what a hematologist needs to know. Haematologica. Mar 2019;104(3):444-455. doi:10.3324/haematol.2018.205526

3. Lang D, Zwerina J, Pieringer H. IgG4-related disease: current challenges and future prospects. Ther Clin Risk Manag. 2016;12:189-99. doi:10.2147/TCRM.S99985

4. Wallace ZS, Naden RP, Chari S, et al. The 2019 American College of Rheumatology/European League against rheumatism classification criteria for IgG4-related disease. Arthritis Rheumatol. Jan 2020;72(1):7-19. doi:10.1002/art.41120

5. Carruthers MN, Khosroshahi A, Augustin T, Deshpande V, Stone JH. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis. Jan 2015;74(1):14-8. doi:10.1136/annrheumdis-2013-204907

6. Malkani RH, Nagral A, Karmakar S, Setia MS. Migratory panniculitis with autoimmune cholangitis and pancreatitis (IgG4- related disease): a rare presentation. Indian J Dermatol. May-Jun 2022;67(3):314. doi:10.4103/ijd.IJD_373_20

7. Kakuchi Y, Yamada K, Suzuki Y, et al. IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis. Intern Med. 2011;50(14):1465-9. doi:10.2169/internalmedicine.50.5239

8. Chiang WY, Liu TT, Huang WT, Kuo MT. Co-existing ligneous conjunctivitis and IgG4-related disease. Indian J Ophthalmol. Jul 2016;64(7):532-4. doi:10.4103/0301-4738.190154

9. Hartman-Adams H, Banvard C, Juckett G. Impetigo: diagnosis and treatment. Am Fam Physician. Aug 15 2014;90(4):229-35.

10. Kaegi C, Wuest B, Schreiner J, et al. Systematic review of safety and efficacy of rituximab in treating immune-mediated disorders. Front Immunol. 2019;10:1990. doi:10.3389/fimmu.2019.01990

11. Chen Y, Cai S, Dong L, Umehara H. Update on classification, diagnosis, and management of immunoglobulin G4-related disease. Chin Med J (Engl). Jan 4 2022;135(4):381-392. doi:10.1097/CM9.0000000000001891