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Neonatal and infantile erythrodermas: a retrospective study of 51 patients
A Pruszkowski1, C Bodemer, S Fraitag
1Service de Dermatologie, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75730 Paris, Cedex 15, France.
Insights
Diagnosing erythroderma in infants is challenging. Key indicators for underlying causes like immunodeficiency include skin induration, alopecia, and failure to thrive, with a poor prognosis noted.
Area of Science:
- Pediatric Dermatology
- Neonatal Medicine
- Clinical Immunology
Background:
- Erythroderma, or generalized skin inflammation, in infants presents diagnostic challenges.
- Identifying the underlying cause is crucial for appropriate management and prognosis.
Purpose of the Study:
- To determine the frequency of various causes of erythroderma in infants.
- To identify clinical and laboratory findings relevant to etiological diagnosis.
Main Methods:
- Retrospective study of 51 infants with exfoliative erythroderma within the first year of life.
- Analysis of clinical features, laboratory findings, and histological data.
Main Results:
- Common causes included immunodeficiency (30%), ichthyosis (24%), Netherton syndrome (18%), and dermatitis (20%).
- Diagnostic clues included congenital onset, skin induration, alopecia, and failure to thrive.
- Histology was valuable for detecting lymphocyte infiltration or necrosis in immunodeficiency.
Conclusions:
- Etiological diagnosis of neonatal erythroderma is difficult; clinical features offer clues but are not definitive.
- Suspect immunodeficiency in severe cases with specific clinical and histological findings.
- The prognosis is poor, with high mortality and persistent severe dermatosis in survivors.
Objective:
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.
Patients:
Fifty-one patients who presented with exfoliative erythroderma during their first year of life were included in this retrospective study.
Setting:
Department of Pediatric Dermatology at a university hospital.
Results:
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.
Conclusions:
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.