Utilization of Rituximab for Refractory Rowell Syndrome

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...

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Main Authors: Sukhraj Singh, Sandra Sheffield, Nisha Chowdhury, Swetha Nuthulaganti, Zareen Vaghaiwalla, Karishma Ramsubeik
Format: Article
Language:English
Published: Wiley 2021-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2021/2727382
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author Sukhraj Singh
Sandra Sheffield
Nisha Chowdhury
Swetha Nuthulaganti
Zareen Vaghaiwalla
Karishma Ramsubeik
author_facet Sukhraj Singh
Sandra Sheffield
Nisha Chowdhury
Swetha Nuthulaganti
Zareen Vaghaiwalla
Karishma Ramsubeik
author_sort Sukhraj Singh
collection DOAJ
description 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.
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spelling doaj-art-e7247b9638a94fefa8529fbaf57de0622025-02-03T05:47:39ZengWileyCase Reports in Rheumatology2090-68892090-68972021-01-01202110.1155/2021/27273822727382Utilization of Rituximab for Refractory Rowell SyndromeSukhraj Singh0Sandra Sheffield1Nisha Chowdhury2Swetha Nuthulaganti3Zareen Vaghaiwalla4Karishma Ramsubeik5Division of Rheumatology, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USADivision of Internal Medicine, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USADivision of Internal Medicine, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USADivision of Internal Medicine, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USADivision of Rheumatology, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USADivision of Rheumatology, Department of Medicine, University of Florida Health Jacksonville, Jacksonville, FL, USARowell 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.http://dx.doi.org/10.1155/2021/2727382
spellingShingle Sukhraj Singh
Sandra Sheffield
Nisha Chowdhury
Swetha Nuthulaganti
Zareen Vaghaiwalla
Karishma Ramsubeik
Utilization of Rituximab for Refractory Rowell Syndrome
Case Reports in Rheumatology
title Utilization of Rituximab for Refractory Rowell Syndrome
title_full Utilization of Rituximab for Refractory Rowell Syndrome
title_fullStr Utilization of Rituximab for Refractory Rowell Syndrome
title_full_unstemmed Utilization of Rituximab for Refractory Rowell Syndrome
title_short Utilization of Rituximab for Refractory Rowell Syndrome
title_sort utilization of rituximab for refractory rowell syndrome
url http://dx.doi.org/10.1155/2021/2727382
work_keys_str_mv AT sukhrajsingh utilizationofrituximabforrefractoryrowellsyndrome
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AT swethanuthulaganti utilizationofrituximabforrefractoryrowellsyndrome
AT zareenvaghaiwalla utilizationofrituximabforrefractoryrowellsyndrome
AT karishmaramsubeik utilizationofrituximabforrefractoryrowellsyndrome