BULLOUS LUPUS, AN UNCOMMON CAUSE OF ATYPICAL ESOPHAGITIS: A CASE REPORT

PV295 / #313 Case Report Poster Topic: AS22 - SLE Heterogeneity Introduction Systemic lupus is a condition that continually reveals its complexities, both systemically and dermatologicaly. Bullous lupus which accounts for less than 5%, poses a true diagnostic and therapeutic challenge. We report a case of bullous lupus with severe esophageal involvement. Case Presentation With Investigation Observation: A 42 years old woman with no significant medical history apart from an esophagitis resistant to treatment for 2 years, presented with dysphagia. An upper digestive endoscopy revealed multiple esophageal strictures with fragile mucous membrane which bleed on contact with the endoscope.This esophagitis was resistant to proton pump inhibitor treatment. Histological examination of the esophageal biopsies suggested a fleshy polyp without evidence of eosinophilic infiltration or tuberculoid granuloma. The immunological assessment (ANA,ANCA,anti-CCP, rheumatoid factor, antiphospholipid antibodies and celia serology) was negative. Additionnally, the patient exhibited fragile skin, oral ulcers and some vesicular and crusty lesions in sun exposed areas. Histological study and direct immunofluorescence of the skin biopsy found an epidermolysis with antiC1q of IgM and IgG type;and C3-C4 complement fraction deposits; which argues for the diagnosis of bullous lupus with lupus esophageal involvement. The patient was placed on immunosuppressive treatment (corticosteroids and azathioprine), with favorable clinical outcomes. Literature Review [1.] Sprow G. Int J Womens Dermatol 2022;8(3);e034. [2.] Smith KN. Dermatol Online J 2023;29(6). Discussion Bullous lupus is an extremely rare entity,and its esophageal involvement complicates diagnosis.Although its therapeutic management may seem straightforward,it remains a significant challenge for practitioners.

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Journal
The Journal of Rheumatology
Published
2025-05-20
DOI
https://doi.org/10.3899/jrheum.2025-0390.pv295
Primary Topic
Eosinophilic Esophagitis
Type
article
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article

BULLOUS LUPUS, AN UNCOMMON CAUSE OF ATYPICAL ESOPHAGITIS: A CASE REPORT

M. Boucelma, Mahdjoubi Amel, Rabahi Ramzi, Bacha Hayette et al.
The Journal of Rheumatology
Eosinophilic Esophagitis
article

BULLOUS LUPUS, AN UNCOMMON CAUSE OF ATYPICAL ESOPHAGITIS: A CASE REPORT

M. Boucelma, Mahdjoubi Amel, Rabahi Ramzi, Bacha Hayette, Boukheris Narimane
article en

Abstract

PV295 / #313 Case Report Poster Topic: AS22 - SLE Heterogeneity Introduction Systemic lupus is a condition that continually reveals its complexities, both systemically and dermatologicaly. Bullous lupus which accounts for less than 5%, poses a true diagnostic and therapeutic challenge. We report a case of bullous lupus with severe esophageal involvement. Case Presentation With Investigation Observation: A 42 years old woman with no significant medical history apart from an esophagitis resistant to treatment for 2 years, presented with dysphagia. An upper digestive endoscopy revealed multiple esophageal strictures with fragile mucous membrane which bleed on contact with the endoscope.This esophagitis was resistant to proton pump inhibitor treatment. Histological examination of the esophageal biopsies suggested a fleshy polyp without evidence of eosinophilic infiltration or tuberculoid granuloma. The immunological assessment (ANA,ANCA,anti-CCP, rheumatoid factor, antiphospholipid antibodies and celia serology) was negative. Additionnally, the patient exhibited fragile skin, oral ulcers and some vesicular and crusty lesions in sun exposed areas. Histological study and direct immunofluorescence of the skin biopsy found an epidermolysis with antiC1q of IgM and IgG type;and C3-C4 complement fraction deposits; which argues for the diagnosis of bullous lupus with lupus esophageal involvement. The patient was placed on immunosuppressive treatment (corticosteroids and azathioprine), with favorable clinical outcomes. Literature Review [1.] Sprow G. Int J Womens Dermatol 2022;8(3);e034. [2.] Smith KN. Dermatol Online J 2023;29(6). Discussion Bullous lupus is an extremely rare entity,and its esophageal involvement complicates diagnosis.Although its therapeutic management may seem straightforward,it remains a significant challenge for practitioners.

The Journal of RheumatologyVol. 52(Suppl 1)
University Frères Mentouri Constantine 1 (DZ), University of Algiers Benyoucef Benkhedda (DZ), University of Algiers 3 (DZ)
Openalex Percentile: Top 80%
Eosinophilic Esophagitis
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