Kotrulja Lena, Murat-Susić Slobodna, Husar Karmela
University Department of Dermatology and Venereology, Sestre milosrdnice University Hospital, Vinogradska 29, HR-10000 Zagreb, Croatia.
Acta Dermatovenerol Croat. 2007;15(3):178-90.
Neonatal and infantile erythroderma is a diagnostic and therapeutic challenge. Numerous underlying causes have been reported. Etiologic diagnosis of erythroderma is frequently difficult to establish, and is usually delayed, due to the poor specificity of clinical and histopathologic signs. Differential diagnosis of erythroderma is a multi-step procedure that involves clinical assessment, knowledge of any relevant family history and certain laboratory investigations. Immunodeficiency must be inspected in cases of severe erythroderma with alopecia, failure to thrive, infectious complications, or evocative histologic findings. The prognosis is poor with a high mortality rate in immunodeficiency disorders and severe chronic diseases such as Netherton's syndrome.
新生儿和婴儿红皮病在诊断和治疗方面具有挑战性。已有众多潜在病因的报道。由于临床和组织病理学体征特异性较差,红皮病的病因诊断常常难以确立,且通常会延迟。红皮病的鉴别诊断是一个多步骤过程,包括临床评估、了解任何相关家族史以及进行某些实验室检查。对于伴有脱发、生长发育迟缓、感染并发症或具有提示性组织学表现的严重红皮病病例,必须检查是否存在免疫缺陷。在免疫缺陷疾病以及诸如 Netherton 综合征等严重慢性疾病中,预后较差,死亡率较高。