Pruszkowski A, Bodemer C, Fraitag S, Teillac-Hamel D, Amoric J C, de Prost Y
Service de Dermatologie, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75730 Paris, Cedex 15, France.
Arch Dermatol. 2000 Jul;136(7):875-80. doi: 10.1001/archderm.136.7.875.
To determine the frequency of the various underlying causes of erythroderma in newborns or infants, as well as which clinical or laboratory findings were relevant for the etiological diagnosis.
Fifty-one patients who presented with exfoliative erythroderma during their first year of life were included in this retrospective study.
Department of Pediatric Dermatology at a university hospital.
On average, the etiological diagnosis was established 11 months after the onset of erythroderma. The underlying causes observed included immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton syndrome (18%), and eczematous or papulosquamous dermatitis (20%). Five patients (10%) had erythroderma of unknown origin. The following parameters were of value in determining the underlying cause of erythroderma: congenital onset, skin induration and the presence of large scaling plaques, alopecia with or without hair dysplasia, evolution, response to topical corticosteroid therapy, presence of infections, and failure to thrive. Histological analysis confirmed the diagnosis in only 19 (45%) of 42 cases. However, it proved of great value for the detection of significant lymphocyte infiltration or keratinocyte necrosis indicating a diagnosis of Omenn syndrome or immunodeficiency. The prognosis was poor in this series: the mortality rate was 16%, and severe dermatosis persisted in 29 (67%) of the survivors.
The etiological diagnosis of neonatal erythroderma is difficult to make; some clinical features may be helpful, but no one feature is characteristic of a cause. An immunodeficiency must be suspected in cases of severe erythroderma with skin induration, severe alopecia, failure to thrive, infectious complications, or evocative histological findings. The prognosis is poor, with a high rate of mortality in immunodeficiency disorders and severe chronic disease in Netherton syndrome and psoriasis.
确定新生儿或婴儿红皮病各种潜在病因的发生率，以及哪些临床或实验室检查结果与病因诊断相关。
51例在出生后第一年出现剥脱性红皮病的患者纳入本回顾性研究。
一所大学医院的儿科皮肤科。
平均而言，红皮病发病后11个月确立病因诊断。观察到的潜在病因包括免疫缺陷（30%）、单纯或复杂鱼鳞病（24%）、Netherton综合征（18%）以及湿疹样或丘疹鳞屑性皮炎（20%）。5例患者（10%）病因不明。以下参数对确定红皮病的潜在病因有价值：先天性发病、皮肤硬结和大的鳞屑斑块、有或无毛发发育异常的脱发、病情演变、对局部皮质类固醇治疗的反应、感染的存在以及生长发育迟缓。组织学分析仅在42例中的19例（45%）中证实了诊断。然而，它对于检测提示Omenn综合征或免疫缺陷诊断的显著淋巴细胞浸润或角质形成细胞坏死具有重要价值。本系列预后较差：死亡率为16%，29例幸存者（67%）中严重皮肤病持续存在。
新生儿红皮病的病因诊断困难；一些临床特征可能有帮助，但没有一个特征是某一病因所特有的。对于伴有皮肤硬结、严重脱发、生长发育迟缓、感染并发症或提示性组织学表现的严重红皮病病例，必须怀疑免疫缺陷。预后较差，免疫缺陷疾病死亡率高，Netherton综合征和银屑病患者有严重慢性疾病。
