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一例副肿瘤性大疱性类天疱疮伴黏膜相关淋巴组织淋巴瘤:一种罕见表现。

A case report of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma: A rare presentation.

机构信息

Department of Cardiology, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh Campus, Sungai Buloh, Selangor, Malaysia.

出版信息

Medicine (Baltimore). 2023 Jan 27;102(4):e32822. doi: 10.1097/MD.0000000000032822.

Abstract

RATIONALE

We report a rare case of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma.

PATIENTS CONCERNS

The patient presented with 5 months' history of generalized skin itchiness, night sweat and loss of weight. The skin manifestations started over the foot and hand area. However, he started to developed tense blisters over the face, trunk and limbs 3 days prior to this admission.

DIAGNOSES

The skin biopsy report showed subepidermal bullae, in which the immunofluorescence findings in keeping with bullous pemphigoid. The peripheral blood immunophenotyping was suggestive of mantle cell lymphoma. Hence, a diagnosis of paraneoplastic bullous pemphigoid associated with mantle cell lymphoma was made.

INTERVENTIONS

The patient was initiated with a cytoreduction chemotherapy.

OUTCOMES

Unfortunately, patient's condition deteriorated further due to neutropenic sepsis and he succumbed after 2 weeks of intensive care.

LESSONS

Bullous pemphigoid associated with mantle cell lymphoma are very rare. The presentation of bullous pemphigoid led to the detection of mantle cell lymphoma. Early diagnosis and appropriate treatment is crucial in managing this aggressive type of the disease. Both, bullous pemphigoid and mantle cell lymphoma had a parallel clinical course which suggests a paraneoplastic phenomenon in this reported case.

摘要

背景

我们报告一例罕见的副肿瘤性大疱性类天疱疮伴套细胞淋巴瘤。

患者情况

患者出现全身皮肤瘙痒、夜间出汗和体重减轻 5 个月。皮肤表现始于足部和手部区域。然而,在本次入院前 3 天,他开始在面部、躯干和四肢出现紧张性水疱。

诊断

皮肤活检报告显示表皮下水疱,免疫荧光检查符合大疱性类天疱疮。外周血免疫表型提示套细胞淋巴瘤。因此,诊断为副肿瘤性大疱性类天疱疮伴套细胞淋巴瘤。

干预措施

患者开始接受细胞减少化疗。

结果

不幸的是,患者因中性粒细胞减少性脓毒症而病情进一步恶化,在重症监护 2 周后死亡。

教训

伴套细胞淋巴瘤的大疱性类天疱疮非常罕见。大疱性类天疱疮的表现导致了套细胞淋巴瘤的检测。早期诊断和适当的治疗对于管理这种侵袭性疾病至关重要。大疱性类天疱疮和套细胞淋巴瘤具有平行的临床过程,提示本例中存在副肿瘤现象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d81/9875989/ec89edde7d78/medi-102-e32822-g001.jpg

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