Neonatal erythroderma: differential diagnosis and management of the “red baby”
- Department of Paediatric Dermatology, Great Ormond Street Hospital for Children, London WC1 3JH, UK
- Dr Harper.
It is essential for paediatricians to recognise erythroderma and distinguish it from eczema. The aim should then be to search for underlying disorders, some of which have a specific treatment. However, for others, much remains to be done until curative treatment will be established for diseases such as Netherton’s syndrome and the ichthyoses.
Erythroderma is defined as an inflammatory skin disorder affecting more than 90% of the body surface.1 It is a reaction pattern of the skin that can complicate many underlying skin conditions at any age. In adults erythroderma may be either drug induced or secondary to pre-existing diseases,2 while in the neonatal period it can be the primary manifestation of several conditions. Table 1 shows the diseases causing congenital or early onset erythroderma. This review outlines the clinical features of these disorders and suggests an approach to differential diagnosis and management.
Many perinatal or early neonatal infectious diseases are associated with a skin eruption; however, only very few manifest as erythroderma.
Staphylococcal scalded skin syndrome (Ritter’s disease, pemphigus neonatorum) is caused by the circulation of exfoliative toxins (ETA, ETB) produced by staphylococci in focal infections such as conjunctivitis, omphalitis, or rhinitis. These exotoxins act as “superantigens” by stimulating large numbers of T cells to release lymphokines (interleukin 2, tumour necrosis factor). Staphylococcal scalded skin syndrome usually manifests in infants and children up to the age of 5 years, but congenital and neonatal cases (following chorioamnionitis) have been described.3 4 The children are irritable and febrile. Within one to two days they develop a generalised macular and subsequently erythrodermic rash, which is accompanied by increased skin tenderness. It precedes the formation of subcorneal blisters, exudation, crusting, and finally generalised exfoliation.
Toxic shock syndrome, similar to staphylococcal scalded skin syndrome, is caused by …