Utilization of Rituximab for Refractory Rowell Syndrome

Abstract

Rowell syndrome describes the occurrence of erythema multiforme-like lesions in patients with cutaneous lesions of lupus erythematosus. The clinical picture of atypical erythema multiforme-like lesions, presence of chilblains, speckled ANA pattern, anti-Ro/SSA, or anti-La/SSB antibodies, and absence of infectious or pharmacologic triggers in a patient with systemic lupus erythematosus are some of the classic clinical and serologic features. Histopathologic and serologic findings can help differentiate this process from erythema multiforme. We present a case of young woman with systemic lupus erythematosus, end-stage renal disease due to lupus nephritis, and a remote history of Steven–Johnson syndrome due to sulfa allergy who presented to the hospital with a recurrent, progressive, targetoid erythematous rash involving more than 60% of her body surface area. Our patient had several hospitalizations in the recent past for this erythematous rash and had failed oral therapy with prednisone 1 mg/kg and hydroxychloroquine. In view of the minimal improvement and increasing severity and patient exhibiting early features of mast cell activation syndrome, the patient was treated with pulse intravenous glucocorticoids followed by rituximab with an excellent response. We highlight a unique case report of progressive Rowell syndrome refractory to standard of care with an excellent response to rituximab.

Rowell Syndrome and SLE Flare: A case report

“…A review of the mentioned cases established that skin symptoms lead to earlier referral of patients, which may be considered a leading indicator for the diagnosis of SLE flareup. According to the mentioned case reports, RS has similar prognoses and treatments to SLE or DLE which occur alone (7)(8)(9)(10). Likewise, our patient responded well to corticosteroids, HCQ, and MMF, and 6 months of follow-up indicated no further recurrence of skin lesions.…”

“…She was treated with prednisolone and HCQ and responded well, which led to the disappearance of her skin lesions and oral ulceration over the next days [7]. RS has even been reported in a patient with end stage renal disease caused by lupus nephritis who had skin rash, pancytopenia, and low C3 and C4 simultaneously; treatment with pulses of methylprednisolone and rituximab maintained the remission of skin disease and cytopenia [8]. Gallo et al reported a unique case of RS.…”

Rowell Syndrome in a Middle-Aged Woman: A Case Report

“…Currently, RS is managed with a similar treatment regimen as LE, including immunomodulatory medications such as methotrexate and rituximab in refractory cases [8][9][10][11]. Most cases of RS are successfully managed with a combination of steroids, azathioprine, antimalarials, dapsone, or cyclosporine [2,3].…”

An impressive case of Rowell syndrome with extensive mucosal involvement successfully treated with anifrolumab

“… 2 While most patients with RS respond well to the aforementioned first-line therapies, reports suggest an urgent need for more effective treatment options for recalcitrant cases. For example, Sigh et al 3 introduced rituximab as an effective treatment for refractory RS. We also greatly appreciate Shope et al 4 reporting a uncommon patient with RS, without mucosal lesions, showing excellent response to anifrolumab, a human monoclonal antibody to the type 1 interferon receptor, after failing first-line treatments and rituximab.…”