Table 2

Neonatal erythroderma: differential diagnosis and management

DiseaseClinical featuresInvestigationsTreatment
Staphylococcal scalded skin syndromePreceding purulent infection; skin tenderness; superficial blisters; positive Nikolsky signSkin swab; assessment of toxin production by
S aureus. Skin biopsy: superficial split (below granular layer), few or no inflammatory cells within bulla or dermis
Intravenous antibiotics (flucloxacillin, amoxycillin/clavulanic acid), contact tracing (carriers of toxigenic strains)
Toxic shock syndromeConcomitant maternal infection; skin tenderness; hypotension/shockSkin swab, assessment of toxin production by
S aureus or Streptococcus pyogenes. Skin biopsy: superficial perivascular and interstitial neutrophilic infiltrates
As above; intravenous immunoglobulins
Congenital cutaneous candidiasisMaternal vaginal candida infection; oral cavity spared; may have paronychia and nail dystrophySkin swab, KOH preparation; pseudohyphae; cultures: urine, blood, CSF. Skin biopsy: pseudohyphae and spores in the corneal layer (PAS stain)Topical (nystatin, miconazole) and oral (nystatin, fluconazole) antimycotics; may need intravenous amphotericin; also eradication of maternal vaginal yeast infection
Omenn’s syndromeLymphadenopathy; sparse hair; unexplained death of previous children; consanguinityEosinophilia; IgE raised; decreased B cells; increased activation markers on T cells (CD25, HLA-Dr, CD45RO); Skin biopsy: activated T cells; eosinophils and histiocytesSupportive care; eventually will need a bone marrow transplant
Graft versus host reactionFever; diarrhoea; antecedent transfusion; immunodeficiencyMixed lymphocyte populations Skin biopsy: basal cell vacuolation; exocytosis, satellite cell necrosis, HLA-Dr + basal keratinocytesUse of irradiated blood products is mandatory; for those with SCID supportive care and bone marrow transplant
Non-bullous ichthyosiform erythrodermaCollodion baby; when shed leaves disseminated ichthyosiform scalingSkin biopsy: hyperkeratosis, acanthosis, minimal lymphocytic infiltrateEmollients
Bullous ichthyosiform erythrodermaSuperficial blistering and erosions; ichthyosiform erythroderma; family history; linear epidermal naevus parents or siblingSkin biopsy: epidermolytic hyperkeratosisEmollients
Netherton’s syndromeDiarrhoea; failure to thrive; atopy; sparse hair, trichorrhexis invaginata (bamboo hair)Hair microscopy shows characteristic features. IgE raised, eosinophilia Skin biopsy: psoriasiform acanthosis, parakeratosis, perivascular lymphocytic infiltrateEmollients, adequate hydration
Conradi-Hünermann syndromeLinear and swirled patterningSkin biopsy: hyperkeratosis, reduced granular layer.x Rays show stippling in infancyEmollients
Holocarboxylase synthetase deficiencyLethargy, coma, apnoeaKetoacidosis, organic aciduria; decreased enzyme activity in leukocytes and fibroblastsOral biotin (5–10 mg/day) (normal daily requirement > 0.1mg/kg/day)
Essential fatty acid deficiencyIchthyosiform erythroderma; wastingBlood fatty acid screenTopical linoleic acid (sunflower seed oil)
CeftriaxoneInfective illness for which this antibiotic has been prescribedNoneSubstitute the antibiotic
Vancomycin“The red man syndrome” sudden hypotension and erythemaNoneReversible on discontinuation
Infantile seborrhoeic dermatitisCradle cap, accentuation in the skin folds of the neck, axillae, and nappy areaMoisturising agents; miconazole-hydrocortisone ointment protective cream nappy area
Atopic dermatitisEncrusted eczema on the scalp and face; generalised eczematous skin; family history for atopyIgE raised; eosinophilia. Skin biopsy: spongiosis, lymphocytes, exocytosisWeak topical steroid; systemic antibiotics if skin infected. Possible cows’ milk allergy
PsoriasisErythematosquamous patches; can be pustular (sterile); may have positive family historySkin biopsy: hyper and parakeratosis, microabscessesBland emollient creams, wet dressings helpful
Pityriasis rubra pilarisSimilar to psoriasis; follicular accentuation skin thickening of palms and soles; may have positive family historySkin biopsy: like psoriasis, follicular hyperkeratosisAlong the same lines as psoriasis
Diffuse mastocytosisDarier’s sign often with blisteringSerum/urine histamine and metabolites. Skin biopsy: mast cell infiltrateH1 and H2 antagonists; oral sodium 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.