Department of Pathology, Hôpital Necker-Enfants Malades, and Université René-Descartes, Paris, France.
Curr Opin Pediatr. 2010 Aug;22(4):438-44. doi: 10.1097/MOP.0b013e32833bc396.
Neonatal erythroderma is a potentially life-threatening condition in neonates less than 1 month old. During the first month of life, erythroderma is generally a presentation of genodermatosis, primary immune deficiency, or, more exceptionally, severe psoriasis, metabolic disease or infection. Atopic erythroderma is observed later in life, usually after the age of 1 month. Rapid determination of the underlying cause is crucial for better management. However, the diagnosis is often a challenge for the clinician and is frequently delayed due to the nonspecific nature of the clinical signs. We summarize the different causes of neonatal erythrodermas and list their clinical, biological, histological, and sometimes genetic characteristics.
Severe erythroderma, typified by early onset, skin induration, severe alopecia and failure to thrive, is immediately suggestive of immunodeficiency or Netherton syndrome. In such cases, an early skin biopsy may be particularly of use in allowing accurate differentiation between these two disorders.
This review outlines the clinical and histological features of these disorders and suggests an approach to their differential diagnosis and management.
新生儿期红皮病是指年龄小于 1 个月的新生儿潜在危及生命的疾病。在生命的第一个月中,红皮病通常是遗传皮肤病、原发性免疫缺陷,或罕见的严重银屑病、代谢疾病或感染的表现。特应性红皮病发生于生命后期,通常在 1 个月后。快速确定潜在病因对更好地管理至关重要。然而,由于临床症状的非特异性,诊断对于临床医生来说常常具有挑战性,并且经常被延迟。我们总结了新生儿期红皮病的不同病因,并列出了它们的临床、生物学、组织学特征,有时还包括遗传学特征。
严重的红皮病,其特征为早期发病、皮肤硬化、严重脱发和生长不良,强烈提示免疫缺陷或 Netherton 综合征。在这种情况下,早期皮肤活检可能特别有助于准确区分这两种疾病。
本综述概述了这些疾病的临床和组织学特征,并提出了一种鉴别诊断和管理的方法。