Case Presentation: A 33 y.o. African American female with a history of systemic lupus erythematosus (SLE) and varicella zoster virus (VZV) infection presented for a progressively worsening left arm and back rash. The patient reported her rash had spread rapidly with increased pain and skin breakdown. She reported associated fever, shortness of breath, and worsening leg swelling. She denied ever receiving a herpes zoster vaccination. She was hospitalized last month for leg swelling and found to have class V lupus nephritis. She was administered pulse-dose IV methylprednisolone which was transitioned to prednisone 60 mg daily and started on mycophenolate mofetil (MMF) 1,500 mg twice daily. She was hypoxic with a chest x-ray showing bilateral pulmonary infiltrates. COVID-19 and HIV tests were negative. Exam showed hemorrhagic vesicular lesions in T3, T4, and T5 dermatomes extending from the back to the right breast with necrosis and purulent discharge. She had similar appearing lesions on her right arm. Patient was placed on IV acyclovir for suspected disseminated VZV. Empiric antibiotics were initiated for bacterial superinfection, which were discontinued after one dose. Prednisone was further tapered down and MMF was held. The lesions improved and hypoxia resolved. The patient was discharged on a reduced dose of MMF 1,000 mg twice daily, a two-month prednisone taper, and valacyclovir 1,000 mg three times a day for one week followed by prophylactic valacyclovir 500 mg twice daily while on steroid therapy.
Discussion: The presented patient had both a disseminated and visceral presentation of VZV as evidenced by several lesions on her upper extremity outside the primary confluence of dermatomes and chest X-ray findings consistent with pneumonia. IV acyclovir was administered once dissemination was recognized resulting in improvement in rash and resolution of VZV pneumonia. Several studies have demonstrated a correlation between SLE and varicella zoster, including increased incidence with lupus nephritis. This is thought to be secondary to reduced CD4 T-cell responses intrinsic to SLE as well as immunosuppression used to treat the disease. According to a 2012 study, MMF both alone and in combination with prednisone caused more cases of varicella zoster than alternative immunosuppressive regimens. With this consideration, our patient was discharged with a reduced dose of MMF while still on a prednisone taper. Bacterial superinfection is a rare manifestation strongly correlated with immunocompromised patients. Although the presented patient was initiated on antibiotics due to the severity of her presentation, improvement with prompt antibiotic discontinuation and viral therapy alone suggested a sole viral origin. Current guidelines recommend VZV infection post-treatment prophylaxis only for patients with solid organ transplantations and hematopoietic stem cell transplantation. Given the presented patient had significant immunosuppression secondary to SLE severity and continued immunosuppressants, she was discharged on prophylaxis with oral valacyclovir while on steroid therapy.
Conclusions: The presented patient with lupus nephritis had a particularly severe presentation of VZV with disseminated and visceral involvement. Limited data exists on the management of immunosuppressants for lupus nephritis complicated by active zoster infection. Further research on the necessity of post-treatment prophylaxis of VZV infection in SLE patients is also required.