186 Arch Dis Child 1998;79:186–191 CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from Neonatal erythroderma: diVerential diagnosis and management of the “red baby” P H Hoeger, J I Harper It is essential for paediatricians to recognise and shock, following transmission of toxigenic erythroderma and distinguish it from eczema. S aureus from an intrauterine infection or at the The aim should then be to search for underly- time of delivery. The extensive skin rash ing disorders, some of which have a specific initially resembles scarlet fever and can develop treatment. However, for others, much remains into erythroderma.5 A similar syndrome can be to be done until curative treatment will be caused by streptoccocal exotoxins.6 established for diseases such as Netherton’s Congenital cutaneous candidiasis is caused syndrome and the ichthyoses. by ascending infection involving the amnion. Erythroderma is defined as an inflammatory Vaginal candidiasis occurs in 20–25% of all skin disorder aVecting more than 90% of the pregnant women. Congenital cutaneous candi- body surface.1 It is a reaction pattern of the skin diasis is characterised by widely scattered mac- that can complicate many underlying skin con- ules, papules, and pustules. Individual skin ditions at any age. In adults erythroderma may lesions may become confluent before evolving be either drug induced or secondary to into exfoliative erythroderma. Associated fea- pre-existing diseases,2 while in the neonatal tures can include paronychia and nail period it can be the primary manifestation of dystrophy.7 Unlike neonatal candidiasis, which several conditions. Table 1 shows the diseases is acquired during passage through the birth causing congenital or early onset erythro- canal, the oral cavity and nappy area are usually derma. This review outlines the clinical fea- spared.8 The course is usually benign, but sys- tures of these disorders and suggests an temic infections (pneumonia and septicaemia) approach to diVerential diagnosis and manage- have been described, especially in premature ment. infants; urine, blood and cerebrospinal fluid cultures are therefore recommended. The very http://adc.bmj.com/ preterm infant is particularly susceptible to Infections developing acquired disseminated cutaneous Many perinatal or early neonatal infectious candidiasis. diseases are associated with a skin eruption; however, only very few manifest as erythro- derma. Immunodeficiency syndromes Because of the protective e ect of maternal Staphylococcal scalded skin syndrome (Rit- V immunity, congenital immunodeficiency syn- on September 29, 2021 by guest. Protected copyright. ter’s disease, pemphigus neonatorum) is dromes are rarely symptomatic at birth. Graft caused by the circulation of exfoliative toxins (ETA, ETB) produced by staphylococci in Table 1 Causes of neonatal erythroderma focal infections such as conjunctivitis, ompha- litis, or rhinitis. These exotoxins act as “super- Infections antigens” by stimulating large numbers of T Staphylococcal scalded skin syndrome Toxic shock syndrome cells to release lymphokines (interleukin 2, Candidiasis tumour necrosis factor). Staphylococcal Immunodeficiency scalded skin syndrome usually manifests in Omenn’s syndrome infants and children up to the age of 5 years, Graft versus host reaction but congenital and neonatal cases (following Ichthyoses 34 Non-bullous ichthyosiform erythroderma chorioamnionitis) have been described. The Conradi-Hünermann syndrome children are irritable and febrile. Within one to Bullous ichthyosiform erythroderma two days they develop a generalised macular Netherton’s syndrome and subsequently erythrodermic rash, which is Metabolic disorders Disorders of biotin metabolism Department of accompanied by increased skin tenderness. It Paediatric Essential fatty acid deficiency Dermatology, Great precedes the formation of subcorneal blisters, Drugs Ormond Street exudation, crusting, and finally generalised Ceftriaxone Hospital for Children, exfoliation. Vancomycin London WC1 3JH, UK Toxic shock syndrome, similar to staphylo- Others Infantile seborrhoeic dermatitis P H Hoeger coccal scalded skin syndrome, is caused by J I Harper Atopic dermatitis Staphylococcus aureus exotoxins (TSST-1, Psoriasis TSST-2).Toxic shock syndrome can present at Pityriasis rubra pilaris Correspondence to: Generalised mastocytosis Dr Harper. or shortly after birth with fever, hypotension, Neonatal erythroderma 187 versus host reaction from maternal engraft- staphylococcal scalded skin syndrome or epi- Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from ment can however occur even during intrauter- dermolysis bullosa. These children later de- ine development. velop typical ichthyosiform hyperkeratosis. Omenn’s syndrome is characterised by exfo- Interestingly, some of the parents of children liative erythroderma with an onset at birth or in with bullous ichthyosiform erythroderma have the early neonatal period. It is associated with had limited disease expression in the form of diVuse alopecia, lymphadenopathy, hepato- linear epidermal naevi with similar histology to splenomegaly, recurrent infections, and failure epidermolytic hyperkeratosis.15 The occur- to thrive.9 Omenn’s syndrome is an autosomal rence of epidermal naevi in a patient with bul- recessive form of severe combined immunodefi- lous ichthyosiform erythroderma has been ciency with particular features: leucocytosis with reported.16 prominent eosinophilia, the presence of in- Netherton’s syndrome is characterised by a creased numbers of (clonal) T cells and triad of generalised exfoliative dermatitis, decreased numbers of B cells, hypogammaglob- sparse hair with trichorrhexis invaginata ulinaemia, and raised IgE.10 In some patients, (“bamboo hair”), and atopic features. It usually maternal or clonal T cells can induce a graft ver- presents at birth as erythroderma (fig 1). sus host reaction. Because of the early paucity of hair, it can take Graft versus host reaction is seen mainly in some time before the diagnosis of Netherton’s children with a T cell immunodeficiency, but syndrome is confirmed, although examination can occur in immunocompetent newborns, as a of eyebrows or eyelashes is often rewarding.17 result of transplacental passage of maternal During their first year of life, patients with lymphocytes during intrauterine11 or postnatal Netherton’s syndrome undergo a period of life (exchange) transfusions.12 The clinical mani- threatening infections, hypernatraemic dehy- festations of a graft versus host reaction include dration, diarrhoea, and failure to thrive, with a fever, a morbilliform rash, which in severe mortality of 30–40% during this period. Later reactions may evolve into an erythroderma, in life, the exfoliative dermatitis tends to persist eosinophilia, lymphocytosis, hepatospleno- and vary in severity, with exacerbations trig- megaly, and lymphadenopathy. The clinical gered by intercurrent illness. Patients are atopic presentation may resemble Omenn’s syn- and often suVer from recurrent angio-oedema drome, but the skin eruption is usually less and urticaria associated with eating certain eczematous in graft versus host reaction. In foods. Apart from raised total IgE and multiple children with congenital immunodeficiency, positive specific IgE reactions, there are no graft versus host reaction occurs within the first consistent immunological abnormalities in two to three weeks of life, but may be present at Netherton’s syndrome.14 birth as erythroderma, after intrauterine Conradi-Hünermann syndrome can present maternofetal transfusion.13 In contrast, in the at birth with erythroderma often in a swirled immunocompetent newborn with small num- pattern and is associated with skeletal (chon- bers of transferred cells, clinical symptomatol- drodysplasia punctata with epiphyseal stip- ogy is minimal and may involve only a transient pling) and eye anomalies (cataracts).14 http://adc.bmj.com/ macular rash. Metabolic disorders Ichthyoses Holocarboxylase synthetase deficiency The hereditary ichthyoses are a large and presents with neonatal erythroderma and heterogeneous group of disorders, which have alopecia. The children are acutely ill with in common rough dry scaly skin. They range ketoacidosis, dehydration, and coma, and most from the barely noticeable scaling of mild ich- of them die within the first week if untreated. In thyosis vulgaris to the large plate-like scales contrast, biotinidase deficiency presents later on September 29, 2021 by guest. Protected copyright. seen in lamellar ichthyosis. Of the diVerent (mean age of onset 3 months) with hypotonia, types of ichthyosis, non-bullous ichthyosiform lethargy, and seizures; the skin lesions resemble erythroderma and bullous ichthyosiform eryth- acrodermatitis enteropathica,18 19 and the alo- roderma manifest at birth with variable degrees pecia is usually patchy. Both types of enzyme of erythroderma. deficiency have previously been described as There are two syndromes associated with “multiple carboxylase deficiency”. Because of ichthyosis that can also feature erythroderma: the lower concentration of biotin in human Netherton’s syndrome and Conradi- milk, breast fed babies with biotinidase defi- Hünermann syndrome. ciency may become symptomatic earlier than Non-bullous ichthyosiform erythroderma is formula fed infants.19 With early biotin substi- characterised by fine white-greyish scales and tution, all skin and neurological symptoms are erythroderma.