Clinicopathological Challenge: Fixed Urticaria-Like Pink Papules in a Patient With Recurrent Fevers and Arthralgias.

IF 3.2 4区 医学 Q1 DERMATOLOGY
Mica C G Williams, Noel Turner, Lourdes Mendez, Jonathan S Leventhal
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引用次数: 0

Abstract

A 67-year-old male with a history of macrocytic anemia presented with widespread pink urticarial papules on his trunk and extremities in the setting of intermittent fevers, lymphadenopathy, arthralgias, and night sweats. Repeat biopsies demonstrated mixed inflammatory infiltrate with eosinophils. Multidisciplinary workup revealed a UBA1 p.Met41Thr variant, confirming a diagnosis of Vacuoles E1 Enzyme X-linked Autoinflammatory Somatic Mutations (VEXAS) syndrome manifesting with urticarial dermatitis.

临床病理挑战:固定荨麻疹样粉红色丘疹患者反复发烧和关节痛。
67岁男性,有大细胞性贫血病史,躯干和四肢广泛出现粉红色荨麻疹丘疹,伴有间歇性发热、淋巴结病、关节痛和盗汗。重复活检显示混合性炎症浸润伴嗜酸性粒细胞。多学科检查发现UBA1 p.Met41Thr变异,确认诊断为液泡E1酶x连锁自身炎症体突变(VEXAS)综合征,表现为荨麻疹皮炎。
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来源期刊
CiteScore
4.70
自引率
2.80%
发文量
476
审稿时长
3 months
期刊介绍: Published monthly, the International Journal of Dermatology is specifically designed to provide dermatologists around the world with a regular, up-to-date source of information on all aspects of the diagnosis and management of skin diseases. Accepted articles regularly cover clinical trials; education; morphology; pharmacology and therapeutics; case reports, and reviews. Additional features include tropical medical reports, news, correspondence, proceedings and transactions, and education. The International Journal of Dermatology is guided by a distinguished, international editorial board and emphasizes a global approach to continuing medical education for physicians and other providers of health care with a specific interest in problems relating to the skin.
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