共 34 条
Neonatal erythroderma
被引:20
作者:
Fraitag, Sylvie
[1
]
Bodemer, Christine
[2
]
机构:
[1] Hop Necker Enfants Malad, Dept Pathol, Serv Anat Pathol, F-75743 Paris 15, France
[2] Univ Paris 05, AP HP, Dept Dermatol,Hop Necker Enfants Malad, Natl Reference Ctr Genodermatosis MAGEC, Paris, France
关键词:
Erythroderma;
ichthyoses;
Netherton syndrome;
Omenn syndrome;
skin biopsy;
CONGENITAL ICHTHYOSIFORM ERYTHRODERMA;
VERSUS-HOST DISEASE;
SEVERE COMBINED IMMUNODEFICIENCY;
COMBINED IMMUNE-DEFICIENCY;
NETHERTON-SYNDROME;
OMENN-SYNDROME;
DIFFERENTIAL-DIAGNOSIS;
MUTATIONS;
GENE;
EXPRESSION;
D O I:
10.1097/MOP.0b013e32833bc396
中图分类号:
R72 [儿科学];
学科分类号:
100202 ;
摘要:
Purpose of review 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. Recent findings 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. Summary This review outlines the clinical and histological features of these disorders and suggests an approach to their differential diagnosis and management.
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页码:438 / 444
页数:7
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