边缘性麻风病患者红皮病的逆转反应1例:加重反应的结果?

Q2 Medicine
Denis Miyashiro, Ana Paula Vieira, Maria Angela Bianconcini Trindade, João Avancini, José Antonio Sanches, Gil Benard
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引用次数: 4

摘要

背景:红皮病的特点是红斑和鳞屑影响90%以上的体表面积。炎症性、肿瘤性和更罕见的感染性疾病可最终发展为红皮病。因此,诊断潜在的疾病对于制定适当的治疗是至关重要的。麻风病是巴西流行的一种慢性传染病。在这里,我们提出一个不寻常的麻风病和逆转反应引起红皮病,我们讨论潜在的免疫机制,这可能有助于全身性皮肤炎症。病例介绍:我们报告一例患者的逆转反应(RR)在边缘性边缘性麻风病表现为红皮病和神经功能障碍。皮肤的组织病理学显示表皮有规律的棘层和海绵状组织,真皮有致密的上皮样肉芽肿,以及成组和分离的杆菌。这种二元性可能反映了从无能/多细菌状态到更有效的免疫和细菌控制状态的转变,这是典型的RR。通过世卫组织的多药治疗加上控制RR的强的松,成功治疗了麻风病;红皮病与这种治疗同时消退。免疫学研究显示IFNγ +原位优势于IL-4+淋巴细胞,IL-17+原位优势于Foxp3+淋巴细胞,提示Th-1/Th-17免疫反应加剧,Th-2和调节性t细胞反应较差。循环treg也减少了。我们假设,抗分枝杆菌免疫反应性的爆发可能引发了强烈的炎症性皮肤病变,最终导致红皮病。结论:本病例报告强调了对红皮病患者进行彻底临床检查以寻找其病因的重要性,并提示强烈且可能不受控制的麻风病RR可能最终导致红皮病的发展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

A case report of erythroderma in a patient with borderline leprosy on reversal reaction: a result of the exacerbated reaction?

Background: Erythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation.

Case presentation: We report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO's multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ + over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma.

Conclusions: This case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.

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来源期刊
BMC Dermatology
BMC Dermatology Medicine-Dermatology
自引率
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期刊介绍: BMC Dermatology is an open access journal publishing original peer-reviewed research articles in all aspects of the prevention, diagnosis and management of skin disorders, as well as related molecular genetics, pathophysiology, and epidemiology. BMC Dermatology (ISSN 1471-5945) is indexed/tracked/covered by PubMed, MEDLINE, CAS, EMBASE, Scopus and Google Scholar.
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