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类天疱疮样鱼鳞病:一种罕见的临床表现。

Pemphigus Foliaceus Mimicking Ichthyosis: A Rare Clinical Presentation.

作者信息

Ahmad Adnan, Al-Sughayer Fatmah, Shaban Manaf, Al-Roomi Shatha

机构信息

Dermatology, Ministry of Health, Sulibekhat, KWT.

Dermatology, Kuwait Institute for Medical Specializations, Sulibekhat, KWT.

出版信息

Cureus. 2024 Nov 28;16(11):e74675. doi: 10.7759/cureus.74675. eCollection 2024 Nov.

Abstract

Pemphigus foliaceus (PF) is an autoimmune blistering disease characterized by the disruption of the epidermal cell adhesion protein desmoglein 1 (DsG1). PF classically presents with superficial erosions or blisters, but can rarely mimic other dermatological conditions, which makes diagnosis challenging. We report the case of a 57-year-old Sri Lankan man with a one-month history of widespread ichthyosis-like plaques and scales which started on his scalp and progressed in a cranio-caudal fashion and were associated with pruritus and few blisters. Histopathology revealed features consistent with PF, and immunofluorescence studies further confirmed the diagnosis of PF. This case highlights the atypical presentation of PF resembling ichthyosis, emphasizing the need to consider autoimmune blistering diseases for unexplained scaling dermatoses. The unusual nature of PF presenting with ichthyosis-like scaly plaques highlights the need for further research. Accurate early diagnosis and treatment can both significantly improve outcomes.

摘要

落叶型天疱疮(PF)是一种自身免疫性水疱病,其特征是表皮细胞黏附蛋白桥粒芯糖蛋白1(DsG1)遭到破坏。PF典型表现为浅表糜烂或水疱,但很少能与其他皮肤病相鉴别,这使得诊断颇具挑战性。我们报告了一例57岁的斯里兰卡男性病例,其有一个月的广泛鱼鳞病样斑块和鳞屑病史,起于头皮,呈头-尾方向进展,并伴有瘙痒和少量水疱。组织病理学显示符合PF的特征,免疫荧光研究进一步确诊为PF。该病例突出了PF类似鱼鳞病的非典型表现,强调对于不明原因的鳞屑性皮肤病需要考虑自身免疫性水疱病。PF以鱼鳞病样鳞屑斑块表现的不寻常性质凸显了进一步研究的必要性。准确的早期诊断和治疗均可显著改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/211a/11681950/b14abc426b52/cureus-0016-00000074675-i01.jpg

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