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. Many patients suVer from deep completely reversible. skin fissures, and some develop flexion contrac- Essential fatty acid deficiency is known to tures. About 90% of patients with non-bullous cause an ichthyosiform erythroderma. This can ichthyosiform erythroderma present as “collo- occur as a result of malabsorption or maldiges- dion babies”14 with a glistening membrane tion, as in severe gastroenteritis and malnutri- resembling sausage skin that envelops the tion, and cystic fibrosis.20 neonate and can produce ectropion, lip ever- sion, and nasal obstruction. Drugs Bullous ichthyosiform erythroderma also Although antibiotics, such as penicillin, ampi- referred to as epidermolytic hyperkeratosis, cillin, aminoglycosides, and cephalosporins, presents with generalised erythema and super- can cause an erythematous macular papular ficial blisters that are frequently mistaken for rash, erythroderma has only been described in 188 Hoeger, Harper Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from

Figure 1 (A) Neonatal erythroderma as a presentation of Netherton’s syndrome. (B) Same boy aged 5 years exhibiting the characteristic facial appearance and short hair of Netherton’s syndrome. (Reproduced from reference 14 with permission of Butterworth Heinemann.)

neonates with ceftriaxone21 and vancomycin.22 and diYcult forms of psoriasis to treat. Psoria- Vancomycin causes a generalised erythema and sis can persist into adulthood and may be com- hypotension owing to histamine release. plicated by pustulosis and early onset arthropathy.25 Pityriasis rubra pilaris is characterised by Others erythematous scaly plaques similar to psoriasis Infantile seborrhoeic dermatitis typically and follicular hyperkeratosis. These can coa- presents during the first month of life. The lesce to larger plaques and become generalised characteristic feature of infantile seborrhoeic as erythroderma. Palmoplantar keratoderma is dermatitis is an inflammatory, yellowish, scal- frequently associated. Congenital erythroder- ing on the scalp (cradle cap) often with mic pityriasis rubra pilaris has been involvement of the skin folds of the neck, axil- described.27 It is inherited as an autosomal lae, and groin. Other forms of presentation are dominant trait and, unlike the acquired forms a psoriasiform appearance or rarely of pityriasis rubra pilaris, tends to run a life erythroderma.23 In a 10 year follow up study, long course. 15% of patients originally presenting with DiVuse cutaneous mastocytosis can present infantile seborrhoeic dermatitis developed at- at birth or in the neonatal period.28 29 The skin opic dermatitis; 28% of children who had pso- is diVusely thickened and may appear doughy. riasiform infantile seborrhoeic dermatitis on There can be numerous orange papules or dif- http://adc.bmj.com/ first presentation and 50% with an erythroder- fuse erythroderma. It can be accompanied by mic presentation went on to develop psoriasis.23 extensive blistering and mimic staphylococcal Although about 18% of all children with skin syndrome.28 Children with congenital dif- atopic dermatitis develop skin symptoms fuse or erythrodermic mastocytosis may have within the first 4 weeks of life,24 the rash is extracutaneous mast cell infiltrates (gastroin- rarely erythrodermic at this age. In young testinal tract, bone, liver, spleen, lymph nodes). infants, the primary lesion of atopic eczema is Accompanying symptoms include diarrhoea, on September 29, 2021 by guest. Protected copyright. frequently vesicular and exudation is common. vomiting, abdominal cramps, wheezing, raised There is a considerable clinical overlap be- temperature, pruritus, flushing, and hypoten- tween manifestation of infantile seborrhoeic sion. Darier’s sign—a wheal and flare reaction dermatitis and atopic dermatitis, and some on rubbing the skin—is positive. Careful moni- authors believe that infantile seborrhoeic der- toring for systemic involvement is mandatory matitis is a variant of atopic dermatitis. Atopic in diVuse cutaneous mastocytosis. It should dermatitis more often involves the face, espe- include full blood count, liver function tests, cially the cheeks, flexural creases of the limbs, bone scan and, if appropriate, gastrointestinal and usually spares the nappy area. In atopic studies. dermatitis itching is usually not apparent until 2 to 3 months of age. Psoriasis at birth or in the neonatal age is Diagnostic approach to neonatal very rare. Only 15 cases of congenital erythro- erythroderma dermic psoriasis have been reported.25 26 It can Neonatal erythroderma is a diagnostic and be similar to non-bullous ichthyosiform eryth- therapeutic challenge. Erythrodermic neonates roderma; distinguishing features between these and infants are frequently misdiagnosed with entities are positive family history and areas of eczema and inappropriate topical steroid treat- unaVected skin in psoriasis, and ectropion in ment can lead to Cushing’s syndrome.30 Delay non-bullous ichthyosiform erythroderma.26 in the establishment of the correct diagnosis More than half of congenital psoriasis patients can be fatal. The diVerential diagnosis of are positive for the HLA antigen B17.26 Most erythroderma is a multistep procedure that cases later develop classic erythematosqua- involves clinical assessment, knowledge of any mous lesions.3 Congenital and neonatal eryth- relevant family history, and certain laboratory rodermic psoriasis are among the most serious investigations (table 2). Neonatal erythroderma 189

Table 2 Neonatal erythroderma: diVerential diagnosis and management Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from

Disease Clinical features Investigations Treatment

Infections Staphylococcal scalded skin Preceding purulent infection; skin Skin swab; assessment of toxin production by Intravenous antibiotics (flucloxacillin, syndrome tenderness; superficial blisters; S aureus. Skin biopsy: superficial split (below amoxycillin/clavulanic acid), contact positive Nikolsky sign granular layer), few or no inflammatory cells tracing (carriers of toxigenic strains) within bulla or dermis Toxic shock syndrome Concomitant maternal infection; Skin swab, assessment of toxin production by As above; intravenous immunoglobulins skin tenderness; hypotension/shock S aureus or Streptococcus pyogenes. Skin biopsy: superficial perivascular and interstitial neutrophilic infiltrates Congenital cutaneous Maternal vaginal candida infection; Skin swab, KOH preparation; pseudohyphae; Topical (nystatin, miconazole) and oral candidiasis oral cavity spared; may have cultures: urine, blood, CSF. Skin biopsy: (nystatin, fluconazole) antimycotics; may paronychia and nail dystrophy pseudohyphae and spores in the corneal layer need intravenous amphotericin; also (PAS stain) eradication of maternal vaginal yeast infection Immunodeficiency Omenn’s syndrome Lymphadenopathy; sparse hair; Eosinophilia; IgE raised; decreased B cells; Supportive care; eventually will need a unexplained death of previous increased activation markers on T cells (CD25, bone marrow transplant children; consanguinity HLA-Dr, CD45RO); Skin biopsy: activated T cells; eosinophils and histiocytes Graft versus host reaction Fever; diarrhoea; antecedent Mixed lymphocyte populations Skin biopsy: Use of irradiated blood products is transfusion; immunodeficiency basal cell vacuolation; exocytosis, satellite cell mandatory; for those with SCID necrosis, HLA-Dr + basal keratinocytes supportive care and bone marrow transplant Ichthyoses Non-bullous ichthyosiform Collodion baby; when shed leaves Skin biopsy: hyperkeratosis, acanthosis, minimal Emollients erythroderma disseminated ichthyosiform scaling lymphocytic infiltrate Bullous ichthyosiform Superficial blistering and erosions; Skin biopsy: epidermolytic hyperkeratosis Emollients erythroderma ichthyosiform erythroderma; family history; linear epidermal naevus parents or sibling Netherton’s syndrome Diarrhoea; failure to thrive; atopy; Hair microscopy shows characteristic features. Emollients, adequate hydration sparse hair, trichorrhexis invaginata IgE raised, eosinophilia Skin biopsy: (bamboo hair) psoriasiform acanthosis, parakeratosis, perivascular lymphocytic infiltrate Conradi-Hünermann Linear and swirled patterning Skin biopsy: hyperkeratosis, reduced granular Emollients syndrome layer. x Rays show stippling in infancy Metabolic Holocarboxylase synthetase Lethargy, coma, apnoea Ketoacidosis, organic aciduria; decreased Oral biotin (5–10 mg/day) (normal daily deficiency enzyme activity in leukocytes and fibroblasts requirement > 0.1mg/kg/day) Essential fatty acid deficiency Ichthyosiform erythroderma; Blood fatty acid screen Topical linoleic acid (sunflower seed oil) wasting Drugs Ceftriaxone Infective illness for which this None Substitute the antibiotic antibiotic has been prescribed Vancomycin “The red man syndrome” sudden None Reversible on discontinuation

hypotension and erythema http://adc.bmj.com/ Others Infantile seborrhoeic Cradle cap, accentuation in the skin Moisturising agents; dermatitis folds of the neck, axillae, and nappy miconazole-hydrocortisone ointment area protective cream nappy area Atopic dermatitis Encrusted eczema on the scalp and IgE raised; eosinophilia. Skin biopsy: spongiosis, Weak topical steroid; systemic antibiotics face; generalised eczematous skin; lymphocytes, exocytosis if skin infected. Possible cows’ milk family history for atopy allergy Psoriasis Erythematosquamous patches; can Skin biopsy: hyper and parakeratosis, Bland emollient creams, wet dressings be pustular (sterile); may have microabscesses helpful

positive family history on September 29, 2021 by guest. Protected copyright. Pityriasis rubra pilaris Similar to psoriasis; follicular Skin biopsy: like psoriasis, follicular Along the same lines as psoriasis accentuation skin thickening of hyperkeratosis palms and soles; may have positive family history

DiVuse mastocytosis Darier’s sign often with blistering Serum/urine histamine and metabolites. Skin H1 and H2 antagonists; oral sodium biopsy: mast cell infiltrate cromoglycate; avoidance of substances with potential for mast cell degranulation (for example, codeine, opiates, aspirin, procaine, radiographic dyes, scopolamine, pancuronium)

CSF, cerebrospinal fluid; SCID, severe combined immunodeficiency.

CLINICAL ASSESSMENT and mastocytosis. Darier’s sign is positive in In the presence of a collodion baby, the diVer- mastocytosis. The Nikolsky sign—the blister can ential diagnosis is virtually restricted to the be propagated within the skin—is evident in sta- hereditary ichthyoses. Once the collodion phylococcal scalded skin syndrome. Alopecia, membrane is shed, skin biopsy will aid in the either total or partial, is obvious in Netherton’s diVerential diagnosis. Children with congenital syndrome, Omenn’s syndrome, and disorders of ichthysiform erythroderma should be moni- biotin metabolism. Lymph node enlargement is tored for associated skeletal, neurological and a characteristic feature of Omenn’s syndrome, ophthalmological abnormalities. but can also be seen in graft versus host reaction Increased skin tenderness, irritability, and and atopic dermatitis. fever should prompt a search for signs of congenital infections. Blistering of the skin is LABORATORY INVESTIGATIONS suspicious of bullous ichthyosiform erythro- DiVerential diagnosis of erythroderma can be derma, staphylococcal scalded skin syndrome, facilitated by a few laboratory tests. Serum IgE 190 Hoeger, Harper

levels are profoundly increased in Netherton’s He was the first to separate this syndrome from Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from and Omenn’s syndromes and mildly increased seborrhoeic and exfoliative dermatitis, respec- in early atopic eczema. Swabs from the skin or tively. However, it has become clear over the other potential sites of infection (eyes, nose, past decade that Leiner’s disease is an umbrella umbilicus, or high vaginal swab from the phenotype rather than a specific entity, and mother) may show growth of S aureus in often applied to babies in whom the known staphylococcal scalded skin syndrome and causes of erythroderma have been excluded. It toxic shock syndrome, and of yeasts in is our opinion that most, if not all, of these congenital cutaneous candidiasis. Serum elec- patients have Netherton’s syndrome, which can trolyte and albumin concentrations should be be diYcult to confirm in the early months of measured because children with erythroderma life. In a study by Glover and colleagues34 of five are at risk of hypernatraemic dehydration and patients with “erythroderma, failure to thrive loss of albumin from enteral and transcutane- and diarrhoea: a manifestation of immunodefi- ous protein losses. A full blood count should be ciency”, one patient went on to develop the supplemented by more detailed immunological typical features of Netherton’s syndrome; of studies if Omenn’s syndrome or graft versus the other four, three had features consistent host reaction are suspected. with Netherton’s syndrome (one died and the other two were lost to follow up) and the fourth HISTOPATHOLOGY had severe combined immunodeficiency. A skin biopsy is essential. As it is very important to establish the diagnosis rapidly, it Presented in part at the 1st annual meeting of the Royal College is advisable to take two or three simultaneous of Paediatrics and Child Health in York, 16–18 April 1997. 31 biopsies from diVerent sites. Samples for rou- 1 Burton JL. Eczema, lichenification, prurigo and erythro- tine histopathology can be formalin fixed, but it derma. In: Champion RH, Burton JL, Ebling FJG, eds. Rook/Wilkinson/Ebling textbook of dermatology. 5th ed. Vol 1. is valuable to have a sample frozen in liquid Oxford: Blackwell Scientific Publications, 1992:537–88. nitrogen for immunohistochemical studies. 2 Sigurdson V, Toonstra J, Hezemans-Boer M, van Vloten WA. Erythroderma. A clinical and follow-up study of 102 Histology and immunohistochemistry can dif- patients, with special emphasis on survival. J Am Acad Der- ferentiate Omenn’s from Netherton’s syn- matol 1996;35:53–7. 32 3 Larrègue M. Les érythrodermies néonatales. Èvolution con- drome and the other ichthyoses. Non-bullous ceptuelle sur cent ans: de 1889 à 1989. Ann Dermatol and bullous ichthyosiform erythroderma are Venereol 1989;116:931–40. 4 Longhead JL. Congenital staphylococcal scalded skin distinguishable on histology. In the graft versus syndrome. Pediatr Infect Dis J 1992;11:413. host reaction, histopathology is valuable in 5 Green SL, La Peter KS. Evidence for postpartum toxic-shock syndrome in a mother-infant pair. Am J Med severe cases, but in milder cases only some of 1982;72:169–72. the findings may be present, and the diagnosis 6 Torres-Martinez C, Mehta D, Butt A, Levin M. Streptococ- cus associated toxic shock. Arch Dis Child 1992;67:126-30. could easily be missed if the histopathologist is 7 Raval DS, Barton LL, Hansen RC, Kling PJ. Congenital not specifically alerted. In blistering diseases, it cutaneous candidiasis: case report and review. Pediatr Der- matol 1995;12:355–8. is helpful to take the biopsy from the border of 8 Chapel TA, Gagliardi C, Nichols W. Congenital cutaneous the blister, including its roof, to facilitate candidiasis. J Am Acad Dermatol 1982;6:926–8.

9 Pupo RA, Tyring SK, Raimer SS, Wirt DP, Brooks EG, http://adc.bmj.com/ assessment of the level of cleavage. Goldblum RM. Omenn´s syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. J Management of children with neonatal Am Acad Dermatol 1991;25:442–6. erythroderma 10 Saint-Basile G, Le Deist F, De Villartay JP, et al. Restricted heterogeneity of T lymphocytes in combined immunodefi- Irrespective of its cause, neonatal erythro- ciency with hypereosinophilia (Omenn’s syndrome). J Clin derma is a potentially life threatening condi- Invest 1991;84:1352–9. 11 Hentschel R, Broecker EB, Kolde G, et al. Intact survival tion. Erythrodermic neonates and infants are at with transfusion-associated graft-versus-host disease risk of hypernatraemic dehydration and hyper- proved by human leukocyte typing of lymphocytes in skin biopsy specimens. J Pediatr 1995;126:61–4. on September 29, 2021 by guest. Protected copyright. pyrexia. Maintaining adequate oral or 12 Ohto H, Anderson KC. Posttransfusion graft-versus-host parenteral fluid intake and monitoring serum disease in Japanese newborns. Transfusion 1996;36:117–23. 13 Alain G, Carrier C, Beaumier L, Bernard J, Lemay M, electrolytes is therefore mandatory. Topical Lavoie A. In utero acute graft-versus-host disease in a applications of emollients (such as white soft neonate with severe combined immunodeficiency. JAm Acad Dermatol 1993;29:862–5. paraYn) hydrate the skin and prevent fissuring. 14 Judge MR, Harper JI. The ichthyoses. In: Harper JI, ed. Blisters and erosions, as seen in bullous ichthy- Inherited skin diseases. The genodermatoses. Oxford: Butter- worth, 1996:69–96. osiform erythroderma, staphylococcal scalded 15 Nazzaro V, Ermacora E, Santucci B, Caputo R. Epidermo- skin syndrome, and mastocytosis, should be lytic hyperkeratosis: generalized form in children from par- ents with systematized linear form. Br J Dermatol treated with a topical astringent, such as 0.01% 1990;122:417–22. potassium permanganate soaks, and systemic 16 Eng AM, Brody P, Rhee HL, Bronson DM. Congenital ich- thyosiform erythroderma and epidermal nevus. Int J antibiotics given if appropriate. As transcutane- Dermatol 1991;30:284–7. ous absorption is profoundly increased,3 addi- 17 Hausser I, Anton-Lambrecht I. Severe congenital general- ized exfoliative erythroderma in newborns and infants: a tives such as salicylic acid or lactic acid should possible sign of Netherton syndrome. Pediatr Dermatol be strictly avoided in the young infant, and 1996;13:183–99. 18 Nyhan WL. Inborn errors of biotin metabolism. Arch topical steroids used cautiously and only after Dermatol 1987;123:1696–98a. the establishment of a diagnosis warranting its 19 Wolf B, Grier RE, Allen RJ, et al. Phenotypic variation in biotinidase deficiency. J Pediatr 1983;103:233–7. use. Table 2 lists specific treatment options. 20 Phillips RJ, Crock CM, Dillon MJ, Clayton PT, Curran A, The term “Leiner’s disease”, which is some- Harper JI. Cystic fibrosis presenting as kwashiorkor with florid skin rash. Arch Dis Child 1993;69:446–8. times used synonymously for infants with 21 Cadoz M, Denis F, Guerma T, Prince-David M, Diop-Mar erythroderma, is confusing and often misun- I. Comparison bacteriologique, pharmacologique et cli- nique de l’amoxycilline et du ceftriaxone dans 300 menin- derstood. In 1908 Carl Leiner, a paediatrician gites purulentes. Pathol Biol Paris 1982;30(6 pt 2):522–5. from Vienna, described a series of young 22 Kucukguclu S, Tuncok Y, Ozkan H, Guven H, Uguz A, Maltepe F. Multiple dose activated charcoal in an acciden- infants with desquamative erythroderma, tal vancomycin overdose. J Toxicol Clin Toxicol 1996;34:83– sparse hair, diarrhoea, and failure to thrive.33 6. Neonatal erythroderma 191

23 Menni S, Piccinno R, Baietta S, CiuVreda A, Scotti L. 29 Smith ML, Orton PW, Chu H, Weston WL. Photochemo- Arch Dis Child: first published as 10.1136/adc.79.2.186 on 1 August 1998. Downloaded from Infantile seborrhoic dermatitis: a seven year follow-up and therapy of dominant, diVuse, cutaneous mastocytosis. Pedi- some prognostic criteria. Pediatr Dermatol 1989;6:13–15. atr Dermatol 1990;7:251–5. 24 Bonifazi E, Meneghini CL. Atopic eczema in the first six 30 Borzykowski M, Grant DB, Wells RS. Cushing’s syndrome months of life. Acta Derm Venereol 1989:144(suppl):20–2. induced by topical steroids used for the treatment of non- 25 Henriksen L, Zachariae H. Pustular psoriasis and arthritis bullous ichthyosiform erythroderma. Clin Exp Dermatol in congenital psoriasiform erythroderma. Dermatologica 1976;1:337–42. 1972;144:12–18. 31 Walsh NM, Prokopetz R, Tron VA, et al. Histopathology in 26 Salleras M, Sanchez-Regana M, Umbert P. Congenital erythroderma: review of a series of cases by multiple erythrodermic psoriasis: case report and literature review. observers. J Cutan Pathol 1994;21:419–23. Pediatr Dermatol 1995;12:231–4. 32 Scheimberg I, Harper JU, Malone M, Lake BD. Inherited 27 GriYths WAD, Leigh IM, Marks R. Disorders of keratiniza- ichthyoses: a review of the histology of the skin. Pediatr tion. In: Champion RH, Burton JL, Ebling FJG, eds. Rook/ Pathol Lab Med 1996;16:359–78. Wilkinson/Ebling textbook of dermatology.5 ed. Vol 2. Oxford: 33 Leiner C. Über Erythrodermia desquamativa, eine eigenar- Blackwell Scientific publishers, 1992:1325–90. tige universelle Dermatose der Brustkinder. Arch Dermatol 28 Oranje AP, Soekanto W, Sukardi A, Vuzevski VD, van der Syphilol 1908;89:163–89. Willigen A, Afiani HM. DiVuse cutaneous mastocytosis 34 Glover MT, Atherton DJ, Levinsky RJ. Syndrome of eryth- mimicking staphylococcal scalded-skin syndrome: report of roderma, failure to thrive and diarrhoea in infancy: a mani- three cases. Pediatr Dermatol 1991;8:147–51. festation of immunodeficiency. Pediatrics 1988;81:66–72.

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