Neonatal erythroderma: differential diagnosis and management
| Disease | Clinical features | Investigations | Treatment |
|---|---|---|---|
| Infections | |||
| Staphylococcal scalded skin syndrome | Preceding purulent infection; skin tenderness; superficial blisters; positive Nikolsky sign | Skin 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 syndrome | Concomitant maternal infection; skin tenderness; hypotension/shock | Skin 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 candidiasis | Maternal vaginal candida infection; oral cavity spared; may have paronychia and nail dystrophy | Skin 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 |
| Immunodeficiency | |||
| Omenn’s syndrome | Lymphadenopathy; sparse hair; unexplained death of previous children; consanguinity | Eosinophilia; IgE raised; decreased B cells; increased activation markers on T cells (CD25, HLA-Dr, CD45RO); Skin biopsy: activated T cells; eosinophils and histiocytes | Supportive care; eventually will need a bone marrow transplant |
| Graft versus host reaction | Fever; diarrhoea; antecedent transfusion; immunodeficiency | Mixed lymphocyte populations Skin biopsy: basal cell vacuolation; exocytosis, satellite cell necrosis, HLA-Dr + basal keratinocytes | Use of irradiated blood products is mandatory; for those with SCID supportive care and bone marrow transplant |
| Ichthyoses | |||
| Non-bullous ichthyosiform erythroderma | Collodion baby; when shed leaves disseminated ichthyosiform scaling | Skin biopsy: hyperkeratosis, acanthosis, minimal lymphocytic infiltrate | Emollients |
| Bullous ichthyosiform erythroderma | Superficial blistering and erosions; ichthyosiform erythroderma; family history; linear epidermal naevus parents or sibling | Skin biopsy: epidermolytic hyperkeratosis | Emollients |
| Netherton’s syndrome | Diarrhoea; 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 infiltrate | Emollients, adequate hydration |
| Conradi-Hünermann syndrome | Linear and swirled patterning | Skin biopsy: hyperkeratosis, reduced granular layer.x Rays show stippling in infancy | Emollients |
| Metabolic | |||
| Holocarboxylase synthetase deficiency | Lethargy, coma, apnoea | Ketoacidosis, organic aciduria; decreased enzyme activity in leukocytes and fibroblasts | Oral biotin (5–10 mg/day) (normal daily requirement > 0.1mg/kg/day) |
| Essential fatty acid deficiency | Ichthyosiform erythroderma; wasting | Blood fatty acid screen | Topical linoleic acid (sunflower seed oil) |
| Drugs | |||
| Ceftriaxone | Infective illness for which this antibiotic has been prescribed | None | Substitute the antibiotic |
| Vancomycin | “The red man syndrome” sudden hypotension and erythema | None | Reversible on discontinuation |
| Others | |||
| Infantile seborrhoeic dermatitis | Cradle cap, accentuation in the skin folds of the neck, axillae, and nappy area | Moisturising agents; miconazole-hydrocortisone ointment protective cream nappy area | |
| Atopic dermatitis | Encrusted eczema on the scalp and face; generalised eczematous skin; family history for atopy | IgE raised; eosinophilia. Skin biopsy: spongiosis, lymphocytes, exocytosis | Weak topical steroid; systemic antibiotics if skin infected. Possible cows’ milk allergy |
| Psoriasis | Erythematosquamous patches; can be pustular (sterile); may have positive family history | Skin biopsy: hyper and parakeratosis, microabscesses | Bland emollient creams, wet dressings helpful |
| Pityriasis rubra pilaris | Similar to psoriasis; follicular accentuation skin thickening of palms and soles; may have positive family history | Skin biopsy: like psoriasis, follicular hyperkeratosis | Along the same lines as psoriasis |
| Diffuse mastocytosis | Darier’s sign often with blistering | Serum/urine histamine and metabolites. Skin biopsy: mast cell infiltrate | H1 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.