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A Retrospective Study of 51 Patients

Anne Pruszkowski, MD; Christine Bodemer, MD; Sylvie Fraitag, MD; Dominique Teillac-Hamel, MD; Jean-Claude Amoric, MD; Yves de Prost, MD, PhD

Arch Dermatol. 2000;136:875-880.

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.

From the Services de Dermatologie (Drs Pruszkowski, Bodemer, Teillac-Hamel, Amoric, and de Prost) and Anatomopathologie (Dr Fraitag), Hôpital Necker–Enfants Malades, Paris, France.

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