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一名年轻女性出现鞍鼻、双侧菜花耳畸形,伴有坏疽性脓皮病样溃疡、空洞性肺部病变、手指坏疽和无脉症。

Saddle-nose and bilateral cauliflower ear deformities with pyoderma gangrenosum-like ulcers, cavitary pulmonary lesions, digital gangrene and pulselessness in a young female.

作者信息

Subhadarshani Sweta, Gupta Vishal, Chahal Anurag, Verma Kaushal K

机构信息

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, Delhi, India.

Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, Delhi, India.

出版信息

BMJ Case Rep. 2017 Jun 15;2017:bcr-2017-220434. doi: 10.1136/bcr-2017-220434.

DOI:10.1136/bcr-2017-220434
PMID:28619741
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5535111/
Abstract

We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made. Overlap with Takayasu's arteritis and granulomatosis with polyangitis, which has been reported rarely in the literature, cannot be excluded.

摘要

我们报告了一名年轻女性,她出现鞍鼻和双侧菜花耳畸形,同时伴有坏疽性脓皮病样溃疡、手指坏疽和无脉症。随后,她被发现有双侧传导性听力损失、角膜混浊、轻度主动脉反流以及肺部空洞性改变和大动脉炎的影像学证据。我们的患者有一系列症状,这对诊断构成了挑战。最终,做出了具有若干不寻常特征的复发性多软骨炎的诊断。与文献中很少报道的Takayasu动脉炎和肉芽肿性多血管炎的重叠情况不能排除。

相似文献

1
Saddle-nose and bilateral cauliflower ear deformities with pyoderma gangrenosum-like ulcers, cavitary pulmonary lesions, digital gangrene and pulselessness in a young female.一名年轻女性出现鞍鼻、双侧菜花耳畸形,伴有坏疽性脓皮病样溃疡、空洞性肺部病变、手指坏疽和无脉症。
BMJ Case Rep. 2017 Jun 15;2017:bcr-2017-220434. doi: 10.1136/bcr-2017-220434.
2
Pyoderma gangrenosum on the nose.鼻子上的坏疽性脓皮病。
Auris Nasus Larynx. 2018 Oct;45(5):1130-1134. doi: 10.1016/j.anl.2018.04.004. Epub 2018 May 10.
3
Takayasu disease presenting as malignant pyoderma gangrenosum in a child with relapsing polychondritis.大动脉炎在一名患有复发性多软骨炎的儿童中表现为恶性坏疽性脓皮病。
J Am Acad Dermatol. 2008 Nov;59(5 Suppl):S84-7. doi: 10.1016/j.jaad.2008.05.010.
4
Multiple cavitary pulmonary nodules in association with pyoderma gangrenosum: case report.多发性空洞性肺结节合并坏疽性脓皮病:病例报告
An Bras Dermatol. 2012 Mar-Apr;87(2):301-4. doi: 10.1590/s0365-05962012000200018.
5
Relapsing polychondritis-associated Takayasu's arteritis.
Folia Med (Plovdiv). 2009 Oct-Dec;51(4):49-52.
6
Rheumatoid arthritis, relapsing polychondritis, and pyoderma gangrenosum evolving into non-Hodgkin's lymphoma.
J Am Osteopath Assoc. 1993 Feb;93(2):240-2, 246-8.
7
Melting corneas with collapsing nose.
Surv Ophthalmol. 1984 Sep-Oct;29(2):148-54. doi: 10.1016/0039-6257(84)90172-3.
8
[Otorhinolaryngologic manifestations of atrophying chronic polychondritis].
Ann Otolaryngol Chir Cervicofac. 1976 Oct-Nov;93(10-11):701-4.
9
Calcified cauliflower ear in relapsing polychondritis.复发性多软骨炎中的钙化菜花耳。
BMJ Case Rep. 2017 Apr 21;2017:bcr-2017-219424. doi: 10.1136/bcr-2017-219424.
10
Saddle nose with sarcoidosis: "A great imitator" of relapsing polychondritis.
Mod Rheumatol. 2018 Nov;28(6):1053-1057. doi: 10.1080/14397595.2016.1193931. Epub 2016 Jun 20.

本文引用的文献

1
Relapsing polychondritis: a review.复发性多软骨炎:综述。
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2
Relapsing polychondritis or ANCA-negative Wegener's granulomatosis?复发性多软骨炎还是抗中性粒细胞胞浆抗体阴性的韦格纳肉芽肿?
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Antinuclear antibodies in relapsing polychondritis.复发性多软骨炎中的抗核抗体。
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Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin.伴有高安动脉炎的坏疽性脓皮病对环孢素治疗有反应。
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A diagnostic dilemma: Wegener's granulomatosis, relapsing polychondritis or both?诊断难题:韦格纳肉芽肿、复发性多软骨炎,还是两者皆有?
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Autoimmune disturbances in relapsing polychondritis and primary alopecia.复发性多软骨炎和原发性脱发中的自身免疫紊乱。
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