Title

Burning Questions on Lupus Rashes

Authors

Rona Yu¹, David Mecham, DO², Michael Loncharich, MD²

1-Medical Student, Uniformed Services University, Bethesda, MD

2-Rheumatology, Internal Medicine, Walter Reed National Military Medical Center, Bethesda, MD

Introduction

Herpes zoster (HZ) is a sporadic reactivation of varicella zoster virus (VZV) that presents as a local, painful vesicular rash. Because the disease is typically limited to older adults, vaccination in younger immunocompromised patients may be overlooked. We report a case of an unvaccinated patient with systemic lupus erythematosus (SLE) who developed disseminated HZ, and highlight the risk of severe presentations in younger patients with autoimmune disorders and the importance of appropriate preventative medicine for immunocompromised patients.

Case Presentation

A 37-year-old woman with SLE manifested by a malar rash, class IV nephritis, leukocytoclastic vasculitis (LCV), and polyarthritis presented with a painful vesicular rash that had developed over the preceding five days. Her SLE medications included hydroxychloroquine (HCQ), belimumab, and mycophenolate mofetil (MMF).

On exam, she had a violaceous vesicular rash on the left lower flank that extended around to the lower abdomen, down the buttocks, and to the lateral thigh spanning the T12-L5 dermatomes. No lesions were found on the nose or eyes. Lab evaluation revealed ESR of 52 mm/hr, C3 of 60.0 mg/dL, C4 of 6.0 mg/dL, double stranded DNA antibodies >300 IU/mL, and spot urine protein/creatinine 0.499.

The patient was admitted for disseminated HZ and treated with intravenous acyclovir (10 mg/kg) for one week before the vesicular lesions crusted, prompting transition to valacyclovir. Belimumab and MMF were discontinued on admission. MMF was restarted after transitioning to valacyclovir and the patient got the first dose of the varicella zoster vaccine within two weeks of discharge.

Discussion

This case illustrates the potential for severe disseminated HZ disease in younger unvaccinated patients on immunosuppressive medications (IS). In SLE, HZ has a prevalence of 30.5%, incidence of 14.3 per 1000 person years, and recurrence rate of 19.5%. Patients with SLE are more susceptible to HZ due to abnormal T cell mediated cytotoxicity and pharmacologic suppression of cellular immunity. Those who do get HZ are more likely to be managed with prednisone, HCQ, MMF, or azathioprine (AZA) than other immunomodulatory medications, though this may be confounded by guidelines prioritizing these agents. Extrapolating from data in patients with rheumatoid arthritis, the RABBIT registry found greater susceptibility to HZ in patients managed with B-cell inhibitors (exposure-adjusted adverse event rate [EAER] 21.5) than those managed with conventional immunomodulatory medications like MMF or AZA (EAER 7.1).

Due to this increased susceptibility, the Centers for Disease Control and Prevention recommends that immunocompromised individuals receive Zoster vaccination as young as age 19, rather than age 50 in healthy adults. The patient was safely vaccinated shortly after resolution of acute infection. Our case serves as a reminder of the importance of preventative medicine for patients with immunocompromising conditions.

References

1. Kwan A, Rayes HA, Lazova T, et al. Herpes zoster in SLE: prevalence, incidence and risk factors. Lupus Sci Med. 2022;9(1):e000574. doi:10.1136/lupus-2021-000574

2. Leuvenink, R., Aeschlimann, F., Baer, W. et al. Clinical course and therapeutic approach to varicella zoster virus infection in children with rheumatic autoimmune diseases under immunosuppression. Pediatric Rheumatology 14, 34 (2016). https://doi.org/10.1186/s12969-016-0095-3

3. Redeker I, Albrecht K, Kekow J, et al. Risk of herpes zoster (shingles) in patients with rheumatoid arthritis under biologic, targeted synthetic and conventional synthetic DMARD treatment: data from the German RABBIT register. Annals of the Rheumatic Diseases 2022;81:41-47.