. 2022 Mar 15;36(7):973–986. doi: 10.1111/jdv.18043

Table 1.

Overview of differential diagnoses in neonatal erythroderma

NON‐SYNDROMAL ICHTHYOSIS

ARCI (including lamellar ichthyosis ©, congenital ichthyosiform erythroderma ©, Harlequin ichthyosis ©), ichthyosis with confetti
Epidermolytic ichthyosis © (including ichthyosis bullosa of Siemens) *
Ichthyosis prematurity syndrome ©
Peeling skin syndrome type B *
Self‐healing collodion * ©
X‐linked recessive ichthyosis * ©

SYNDROMAL ICHTHYOSIS

Netherton syndrome ©
Sjögren‐Larsson syndrome ©
Chanarin‐Dorfman syndrome
Conradi‐Hünermann‐Happle syndrome * ©
Keratitis‐ichthyosis‐deafness syndrome (KID), KID‐AR, and ichthyosis hystrix of Curth‐Macklin ©
Trichothiodystrophy ©
IFAP syndrome *

ECTODERMAL DYSPLASIA

Ankyloblepharon‐Ectodermal defects‐Cleft lip/palate syndrome (AEC)

METABOLIC DISORDERS

Multiple carboxylase deficiency (Holocarboxylase synthetase deficiency *and biotinidase deficiency *)
Amino Acid disorders (Methylmalonic acidemia, propionic acidemia and maple syrup urine disease) *
Urea cycle disorders (citrullinemia *)
Menkes disease

INFECTIONS

Staphylococcal scalded skin syndrome
Congenital cutaneous candidiasis

Reference(s)

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IMMUNODEFICIENCIES

Omenn syndrome
SCID
Wiskott‐Aldrich syndrome (WAS) *
Immunodysregulation, polyendocrinopathy and enteropathy. X‐linked, syndrome (IPEX) *
Maternal graft‐versus‐host disease
Severe dermatitis, multiple allergies, and metabolic wasting syndrome (SAM), SAM‐like phenotype *
Di George Syndrome *
Selective IgA deficiency *
Gaucher Syndrome type 2 ©
AD/AR‐ Hyper IgE syndrome*
X‐linked agammaglobulinemia*
CVID*

DRUGS

Stevens‐Johnson syndrome *
Toxic epidermal necrolysis *
Drug‐induced hypersensitivity syndrome (vancomycin , phenytoin and ceftriaxon)
Toxic shock syndrome *

OTHER

Atopic dermatitis *
Psoriasis
Seborrheic dermatitis *
Pityriasis rubra pilaris *
Diffuse cutaneous mastocytosis
COFS syndrome
Kindler EB
After cow milk consumption
Scabies
Hemophagocytic lymphohistiocytosis *
Congenital lues *
Acrodermatitis enteropathica*

Reference(s)

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AD, autosomal dominant; AR, autosomal recessive; ARCI, Autosomal recessive congenial ichthyosis; COFS, Cerebro‐oculo‐facio‐skeletal syndrome; CVID, combined variable immunodeficiency; EB, epidermolysis bullosa; IFAP, Ichthyosis Follicularis Atrichia and Photophobia; SCID, severe combined immunodeficiency.