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首页> 外文期刊>Journal of cutaneous pathology >Koebnerization of Hailey-Hailey disease into a cutaneous drug eruption of acute generalized exanthematous pustulosis associated with systemic symptoms
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Koebnerization of Hailey-Hailey disease into a cutaneous drug eruption of acute generalized exanthematous pustulosis associated with systemic symptoms

机译：将Hailey-Hailey病Koebnerization转化为与全身症状相关的急性全身性皮疹性皮疹的皮肤药疹

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摘要

We describe a 65-year-old Caucasian female with well-controlled Hailey-Hailey disease (HHD) who developed acute generalized exanthematous pustulosis (AGEP) with severe systemic symptoms. Despite sparing of the patient's intertriginous skin, histopathologic evidence of HHD was observed in all biopsies, suggestive of a unique koebernization phenomenon of HHD to areas of cutaneous drug eruption. While internal organ involvement is less commonly reported in AGEP, there are an increasing number of patients with signs and symptoms suggestive of an AGEP/drug reaction with eosinophilia and systemic symptoms (DRESS) spectrum of cutaneous drug disorders. Early diagnosis of patients with AGEP and systemic symptoms is critical so that these patients may receive prompt and aggressive systemic therapy to decrease the risk of end organ damage and improve overall morbidity and mortality.
机译：我们描述了一个65岁的白种女性，她患有可控的Hailey-Hailey病（HHD），可发展为严重全身症状的急性全身性皮疹性脓疱病（AGEP）。尽管保留了患者的三叉皮肤，但在所有活检组织中均观察到HHD的组织病理学证据，这提示HHD在皮肤药疹区域具有独特的koebernization现象。虽然在AGEP中较少报道内脏器官受累，但越来越多的患者出现体征和症状，提示其发生嗜酸性粒细胞增多和皮肤药物异常的全身症状（DRESS）频谱的AGEP /药物反应。患有AGEP和全身症状的患者的早期诊断至关重要，因此这些患者可以接受迅速而积极的全身治疗，以降低终末器官损害的风险并提高总体发病率和死亡率。

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