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CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 15 - 2024 |
doi: 10.3389/fimmu.2024.1419054
This article is part of the Research Topic Inflammatory Disorders of the Oral Mucosa: from Stem Cell Biology to Clinical Management View all 5 articles
Case report: bullous pemphigoid combined with Sjögren's syndrome complicated by central nervous system infection
Provisionally accepted- Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, China
Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease in humans, characterized by tense blisters, erosions, urticarial lesions, and itching on normal or erythematous skin. Many autoimmune diseases are considered comorbidities of BP, but clinical case reports of BP complicated by Sjögren's syndrome are very scarce. Furthermore, cases of central nervous system infection secondary to both autoimmune diseases are even rarer. Case presentation: we report a 74-year-old woman diagnosed with bullous pemphigoid, who showed relief of active lesions after treatment with methylprednisolone and dupilumab injections. However, she was admitted for pulmonary infection during which she was diagnosed with Sjögren's syndrome (SS). Subsequently, the patient developed altered consciousness, indicating a central nervous system infection. Adjustment of steroid dosage and aggressive antimicrobial therapy led to alleviation of symptoms.The coexistence of autoimmune subepidermal blistering diseases and SS is rare. The role of SS in the pathogenesis of skin lesions is unclear, and the relationship between these blistering diseases and SS remains elusive. Further research is needed to determine whether there are common pathological mechanisms between the two conditions. 【Keywords】 Bullous pemphigoid; Sjogren's syndrome; Pathological mechanisms; Autoimmune diseases; Central nervous system infection 【Introduction】 Bullous pemphigoid (BP) is the most common autoimmune blistering disease, primarily affecting the elderly, with peak onset around 80 years of age, and no significant gender difference [1]. Clinical manifestations mainly include erythematous urticarial plaques, blisters, and intense itching. It is induced by autoantibodies against two dermal-epidermal junction proteins, BP180 and BP230. Binding of autoantibodies leads to complement activation, inflammatory reactions, degranulation of mast cells, accumulation of neutrophils and eosinophils, and release of proteases cleaving BP180, resulting in blister formation [2]. The pathogenesis of BP is largely unknown, but previous cases have been associated with specific "potential triggers" such as drugs [3][4], physical factors, vaccines[5], infections, and transplants. The gold standard for BP
Keywords: Bullous pemphigoid, Sjogren' s syndrome, Pathological mechanisms, Autoimmune Diseases, Central nervous system infection
Received: 17 Apr 2024; Accepted: 26 Aug 2024.
Copyright: © 2024 Chen, Chen, Long, Ding, Li and Zhi. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Xing-Yue Chen, Hospital of Chengdu University of Traditional Chinese Medicine, Chengdu, China
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