Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page Small font size Default font size Increase font size Users Online: 270

 Table of Contents  
Year : 2020  |  Volume : 21  |  Issue : 1  |  Page : 15-21

Neonatal and infantile erythroderma revisited

1 Department of Dermatology, AIIMS, New Delhi, India
2 Department of Dermatology, Maulana Azad Medical College, New Delhi, India

Date of Submission11-Sep-2019
Date of Decision29-Sep-2019
Date of Acceptance09-Oct-2019
Date of Web Publication24-Dec-2019

Correspondence Address:
Dr Rashmi Sarkar
Department of Dermatology, Maulana Azad Medical College, New Delhi - 110 002
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_93_19

Rights and Permissions

Erythroderma is the term applied to any inflammatory skin disease affecting more than 90% of the body surface. The incidence of neonatal and infantile erythroderma was 0.11% in an Indian study. There are various causes of neonatal and infantile erythroderma such as congenital, metabolic, papulosquamous, eczematous, drug induced, infections, autoimmune diseases, and malignancy but it is quite difficult to establish the etiology in early stage of life due to less specific clinical, biochemical and histological findings as compared to adults. Proper history, examination, skin biopsy, and various other relevant investigations (based on the differential diagnoses) are imperative to the diagnosis of neonatal and infantile erythroderma. Ruling out immunodeficiency in a child with failure to thrive is important. Since erythroderma is a dermatological emergency, its wise management is mandatory. Vitals, input-output, serum electrolytes, etc., are to be precisely monitored. Barrier nursing is of utmost importance in such cases. Any complication is to be dealt with, and specific treatment of particular cause of childhood and infantile erythroderma is to be done.

Keywords: Dermatological emergency, inflammatory skin, neonatal and infantile erythroderma

How to cite this article:
Swarnkar B, Sarkar R. Neonatal and infantile erythroderma revisited. Indian J Paediatr Dermatol 2020;21:15-21

How to cite this URL:
Swarnkar B, Sarkar R. Neonatal and infantile erythroderma revisited. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Aug 7];21:15-21. Available from: http://www.ijpd.in/text.asp?2020/21/1/15/273846

  Introduction Top

Erythroderma or exfoliative dermatitis is the term applied to any inflammatory skin disease affecting more than 90% of the body surface.[1] It is a reaction pattern of the skin manifested by erythema and scaling that can complicate many underlying skin conditions at any age. It is a less common entity in the pediatric patients.[2]

The incidence of neonatal and infantile erythroderma was 0.11% in a study done by Sarkar et al. in Delhi.[3]

In adults, erythroderma may be due to drugs or secondary to preexisting dermatoses, while in the neonatal period it can be the primary manifestation of several conditions such as immunodeficiency and ichthyosiform syndrome.[4] The etiology of neonatal and infantile erythroderma is difficult to find and is often delayed due to the less specific clinical, biochemical and histological signs. Moreover, the causes of erythoderma in this age group are quite distinctive.[5]

It is imperative for dermatologists as well as pediatricians to recognize erythroderma, its consequences and differentiate it from benign erythemas of childhood, and to prevent its mismanagement.

  Classification Top

It can be classified based on the following:

  1. Duration of disease

    • Acute (few days)
    • Chronic.

  2. Age of onset [6]

    • Congenital

      • Ichthyosis
      • Primary immunodeficiency syndrome (PIDS).

    • Neonatal (<1 month)

      • Ichthyosis
      • PIDS
      • Psoriasis
      • Metabolic disease.

    • Infantile (>1 month–1 year)

      • PIDS
      • Seborrhoeic dermatitis
      • Papulosquamous disorders- psoriasis, pityriasis rubra pilaris (PRP)
      • Atopic dermatitis
      • Ichthyosis
      • Drug-induced
      • Metabolic/nutritional
      • Cutaneous T-cell lymphoma
      • Rarely - Kawasaki disease, dermatomyositis, sarcoidosis, pemphigus foliaceus.

  3. Vesicles/bullae formation [6]

    • Bullous erythroderma

      • Staphylococcal scalded skin syndrome (SSSS)
      • Bullous ichthyosis
      • Diffuse cutaneous mastocytosis.

    • Scaling and exfoliative erythroderma

      • Keratinization disorders
      • Immune deficiencies
      • Metabolic diseases.

  4. Etiology [4],[5],[7],[8],[9],[10],[11]

    • Ichthyosis

      • nonbullous ichthyosiform erythroderma (NBIE)
      • Bullous ichthyosiform erythroderma (BIE)
      • Netherton's syndrome
      • Conradi–Hunermann syndrome (CHS)
      • Sjogren–Larsson syndrome (SLS)
      • Erythrokeratoderma
      • Lamellar ichthyosis [Figure 1]
      • Trichothiodystrophy (TTD)
      • Keratitis Ichthyosis Deafness (KID) syndrome
      • Neutral lipid storage disease with ichthyosis.

    • Transient neonatal dermatoses

      • Miliaria
      • Erythema toxicum neonatorum.

    • Infections

      • SSSS
      • Scarlet fever
      • Neonatal candidiasis
      • Toxic shock syndrome (TSS)
      • Congenital herpes simplex virus (HSV) infection
      • Syphilis.

    • Infestations

      • Norwegian scabies.

    • Immunodeficiency

      • Omenn's syndrome
      • Severe combined immunodeficiency syndrome (SCID)
      • Graft versus host disease
      • Wiskott–Aldrich syndrome
      • Di Georges syndrome.

    • Drug-induced

      • Boric acid toxicity
      • Ceftriaxone
      • Ampicillin, amoxicillin
      • Vancomycin
      • Antiepileptics
      • Sulfonamides
      • Topical tar
      • Thioacetazone
      • Captopril
      • Cimetidine
      • Antitubercular drugs
      • Homeopathic and indigenous drugs.

    • Metabolic/nutritional

      • Disorders of biotin metabolism
      • Multiple carboxylase deficiency
      • Essential fatty acid deficiency
      • Urea cycle disorders (argininosuccinic aciduria, citrullinaemia)
      • Renal failure
      • Kwashiorkor
      • Acrodermatitis enteropathica
      • Cystic fibrosis
      • Leiner's disease.

    • Eczematous disorders

    • Papulosquamous disorders

      • Psoriasis
      • PRP.

    • Malignancies

      • Diffuse cutaneous mastocytosis
      • Cutaneous T-cell lymphoma.

    • Autoimmune

      • Dermatomyositis
      • Kawasaki disease
      • Sarcoidosis
      • Pemphigus foliaceus.

    • Ectodermal dysplasias
    • Idiopathic.
Figure 1: An infant with autosomal recessive ichthyosiform erythroderma (lamellar type)

Click here to view
Figure 2: An infant with erythroderma secondary to seborrheic dermatitis (back view)

Click here to view
Figure 3: An infant with erythroderma secondary to seborrheic dermatitis (front view)

Click here to view

  Etiology Top

In a retrospective study done in France by Pruszkowski et al., on 51 cases of erythroderma in neonates and infants, excluding those with blistering, the various underlying causes were: immunodeficiency - 30%, ichthyosis (simple and complex) - 24%, Netherton syndrome - 18%, eczematous and papulosquamous dermatitis - 20%, and erythroderma of unknown etiology-10%[12] [Table 1] shows causes of childhood erythroderma in studies done in India.
Table 1: Causes of childhood erythroderma in India

Click here to view

  Pathogenesis Top

The pathogenesis of erythroderma appears complex. It develops secondary to an intricate interaction of cytokines and cellular adhesion molecules like interleukin 1, 2, intercellular adhesion molecule, and the tumor necrosis factor (TNF). These interactions result in a florid increase in the epidermal cell turnover rate and accelerated mitosis resulting in excessive scaling and erythema.[15],[16],[17],[18] It manifests as a excessive loss of epidermal material along with protein, iron, and folate.[17] In addition to these basic changes, all above mentioned diseases have their own specific pathogenesis.

  Approach Top

1. History [4],[5],[7],[8],[9],[10],[11],[19]

  • Family history-BIE, psoriasis, PRP, Omenn's syndrome, Netherton's syndrome, atopy
  • History of consanguinity-Ichthyosis, Netherton syndrome, primary immunodeficiency disorder
  • Congenital onset-ichthyosis, infections, immunodeficiency disorders
  • Recurrent infection and diarrhea-immunodeficiency disorders
  • Neurological complaints (Ichthyosiform syndromes, biotinase deficiency)
  • Blood transfusion-GVHR
  • Preceding purulent infection-SSSS
  • Concomitant infection in mother-TSS, congenital cutaneous candidiasis (CCC)
  • Failure to thrive - Netherton syndrome, immunodeficiency diseases, metabolic disorders
  • Diarrhea - GVHR, Netherton syndrome, Omenn syndrome
  • Periorificial dermatitis-metabolic and nutritional disorders
  • Blisters-SSSS, mastocytosis, BIE
  • Collodion baby - NBIE and other ichthyosis
  • Fever-SSSS, GVHR, TSS
  • Atopy-Netherton syndrome, atopic dermatitis
  • Presence of linear epidermal nevus in family members - BIE
  • H/O unexplained death in previous children - Omenn's syndrome.

2. Examination [4],[5],[7],[8],[9],[10],[11],[19]

  1. General

    • Shock-TSS, toxic epidermal necrolysis (TEN)
    • Lymphadenopathy – Omenn's syndrome, GVHR, atopic dermatitis
    • Hepatosplenomegaly-Omenn's syndrome, GVHR.

  2. Systemic

    • Ophthalmological-cataract - CHS, neutral lipid storage disease, fundus-glistening dots-SLS
    • Auditory-KID syndrome, neutral lipid storage disease
    • Respiratory - cystic fibrosis
    • CNS-TTD (spasticity, mental retardation).

  3. Mucocutaneous

    • Type of scales (ichthyosiform or fine scales)
    • Spared areas - PRP
    • The presence of specific lesion (keratotic follicular papules-PRP, well-defined erythematous scaly plaques-psoriasis, bulla, and erosions)

    • Skin induration-immunodeficiency, atopic dermatitis, neuroichthyosis, generalized cutaneous mastocytosis
    • Darier's sign-mastocytosis
    • Positive Nikolsky's sign-SSSS, boric acid toxicity
    • Skin tenderness - SSSS, TEN, TSS
    • Distribution (flexor or extensor)
    • Sparing of napkin area and axillae - Atopic dermatitis
    • Nail changes-paronychia and dystrophy in CCC
    • Alopecia-Omenn's syndrome, GVHR, Netherton's syndrome, Biotin metabolism disorders, citrullinemia, trichothiodystrophy (brittle hair)
    • Hair dysplasia - Netherton syndrome (bamboo hair-trichorrhexis invaginata, nodosa)
    • Mucosal examination
    • Palmoplanter thickening - PRP
    • Swirled pattern of erythroderma - CHS
    • Ectrodactyly, cleft lip-ectodermal dysplasia.

3. Investigations [4],[5],[7],[8],[9],[10],[11],[19]

  • Skin biopsy - It is essential, and as in adults, it is advisable to take 2–3 specimens simultaneously from different sites.

Findings are mentioned in [Flow Chart 1].

  • Swabs from skin, eyes, nose, and umbilicus – SSSS, TSS, CCC
  • Culture of urine, blood, and CSF - candidiasis
  • KOH mount - candidiasis
  • Tzanck smear - Pemphigus foliaceus, HSV infection
  • Wet mount - Sarcoptes mite
  • Dermatoscopy - Netherton syndrome
  • Hair microscopy - Netherton syndrome (bamboo hair), Trichothyodystrophy (alternate dark and light bands-tiger skin pattern)
  • High vaginal swab from mother -Staphylococcus aureus or CCC.
  • Complete blood count - Anemia-Cystic fibrosis, malnutrition

    • Leukocytosis - SSSS, TSS
    • Eosinophil count – It is markedly increased in Omenn syndrome and Netherton syndrome. Mildly increased in atopic dermatitis
    • Thrombocytopenia - Wiskott Aldrich syndrome.

    • Serum IgE and other immunoglobulins – Omenn syndrome, Netherton's syndrome, atopic dermatitis
    • Complement level - Leiner's disease
    • T and B lymphocytes count - Immunodeficiency syndromes
    • Zinc and alkaline phosphate levels - Acrodermatitis enteropathica
    • Assays of essential fatty acids, amino acids, holocarboxylase and biotinidase-to rule out metabolic disorders
    • Sweat chloride levels-cystic fibrosis
    • Holocarboxylase synthetase activity in leukocytes and fibroblasts-hydroxysteroid dehydrogenase
    • Blood for fatty acid levels-essential fatty acid deficiency
    • Genetic analysis for SPINK5 - Netherton syndrome
    • X-ray long bones - CHN
    • X-ray chest-absent thymic shadow - SCID
    • Histamine and its metabolite levels, tryptase in serum or urine-mastocytosis
    • Serum electrolytes-hypernatremic dehydration
    • Serum albumin
    • HLA B17-positive in congenital psoriasis
    • Chromatography-blood and urine-carboxylase deficiency and urea cycle disorders
    • Audiometry - KID syndrome
    • Capillary blood gas analysis-ketoacidosis (holocarboxylase synthetase deficiency) [Flow Chart 2].

  Treatment Top

General measures

Temperature, pulse, respiratory rate, blood pressure, and input-output need to be strictly monitored. Electrolyte, metabolic acidosis is to be looked for and managed carefully. Adequate application of bland emollients, wet dressings are helpful in decreasing scaling and fissuring. 0.01% potassium permanganate soaks are used over erosions for its astringent, antibacterial, and antifungal effects. Severe complications such as septicemic infections, hypoalbuminemia, hyperpyrexia, hypernatremic dehydration, and cardiac failure should be managed timely.[4],[5],[7],[8],[9],[10],[11],[19]

Specific measures

Treatment of underlying diseases such as antibiotics, antifungals, and antiscabicidal for bacterial infections, candidiasis, and scabies, respectively. Atopic dermatitis and drug-induced erythroderma may need a short course of oral corticosteroids. Drug-induced cases also require withdrawal of offending drug. Psoriasis, ichthyosis may need oral retinoids while immunosuppressants are required for the management of dermatomyositis, pemphigus foliaceus, and sarcoidosis. Immunodeficiencies are treated with bone marrow transplantation.[4],[5],[7],[8],[9],[10],[11],[19]

  Recent Advances Top

  • Oral liarozole, a retinoic acid metabolism-blocking agent, an alternative to systemic retinoid therapy, can be given in patients with lamellar ichthyosis [20]
  • Water-in-oil emulsion of 10% N-acetyl cysteine in combination with 5% urea topically for lamellar ichthyosis, epidermolytic ichthyosis which can present as erythroderma [21],[22]
  • Being relatively new, the experience of biologics in children is very less. Recommendations of Italian expert group for the treatment of severe psoriasis including erythrodermic psoriasis exist. This group has recommended adalimumab as a 1st-line treatment option for severe psoriasis in children >4 years of age, and etanercept and ustekinumab as the 2nd-line treatment modalities for children >6 years and >12 years of age, respectively.[23]

  Conclusion Top

Neonatal erythroderma is a potentially life-threatening condition and its clinical care and prognosis are related to its underlying causes. Many a times, it is difficult to reach an aetiological diagnosis on the basis of clinical information alone and additional measures, especially skin biopsy, may be very helpful. There is a need of multidisciplinary approach for the management of such patients by dermatologists and pediatricians.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Burton JL, Holden CA. Eczema, lichenification and prurigo. In: Champion RH, Burton JL, Burns DA, Breathnach SM, editors. Text Book of Dermatology. Oxford: Blackwell Scientific Publications; 1998. p. 673-8.  Back to cited text no. 1
King LE Jr. Erythroderma. Who, where, when, why, and how. Arch Dermatol 1994;130:1545-7.  Back to cited text no. 2
Sarkar R, Basu S, Sharma RC. Neonatal and infantile erythrodermas. Arch Dermatol 2001;137:822-3.  Back to cited text no. 3
Hoeger PH, Harper JI. Neonatal erythroderma: Differential diagnosis and management of the “red baby”. Arch Dis Child 1998;79:186-91.  Back to cited text no. 4
Sarkar R. Neonatal and infantile erythroderma: “The red baby”. Indian J Dermatol 2006;51:178-82.  Back to cited text no. 5
  [Full text]  
Fraitag S, Bodemer C. Neonatal erythroderma. Curr Opin Pediatr 2010;22:438-44.  Back to cited text no. 6
Sarkar R, Garg VK. Erythroderma in children. Indian J Dermatol Venereol Leprol 2010;76:341-7.  Back to cited text no. 7
[PUBMED]  [Full text]  
Sehgal VN, Srivastava G. Erythroderma/generalized exfoliative dermatitis in pediatric practice: An overview. Int J Dermatol 2006;45:831-9.  Back to cited text no. 8
Dhar S, Banerjee R, Malakar R. Neonatal erythroderma: Diagnostic and therapeutic challenges. Indian J Dermatol 2012;57:475-8.  Back to cited text no. 9
[PUBMED]  [Full text]  
Kotrulja L, Murat-Susić S, Husar K. Differential diagnosis of neonatal and infantile erythroderma. Acta Dermatovenerol Croat 2007;15:178-90.  Back to cited text no. 10
Ott H, Hütten M, Baron JM, Merk HF, Fölster-Holst R. Neonatal and infantile erythrodermas. J Der Dtsch Dermatol Ges 2008;25:6 Suppl 12:1070-86.  Back to cited text no. 11
Pruszkowski A, Bodemer C, Fraitag S, Teillac-Hamel D, Amoric JC, de Prost Y. Neonatal and infantile erythrodermas: A retrospective study of 51 patients. Arch Dermatol 2000;136:875-80.  Back to cited text no. 12
Sarkar R, Sharma RC, Koranne RV, Sardana K. Erythroderma in children: A clinico-etiological study. J Dermatol 1999;26:507-11.  Back to cited text no. 13
Kalsy J, Puri K. Erythroderma in children: Clinico-etiological study from Punjab. Indian J Paediatr Dermatol 2013;14:9-12.  Back to cited text no. 14
  [Full text]  
Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative dermatitis: A synopsis. Int J Dermatol 2004;43:39-47.  Back to cited text no. 15
Wilson DC, Jester JD, King LE Jr. Erythroderma and exfoliative dermatitis. Clin Dermatol 1993;11:67-72.  Back to cited text no. 16
Kimgai-Asadi A, Freedberg IM. Exfoliative dermatitis. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Fitzpatrick's Dermatology in General Medicine. 4th ed. New York: Mc Graw-Hill; 1993. p. 527-30.  Back to cited text no. 17
Hendrix JD Jr., Greer KE. Erythrokeratodermia variabilis present at birth: Case report and review of the literature. Pediatr Dermatol 1995;12:351-4.  Back to cited text no. 18
Sarkar R, Garg S, Garg VK. Neonatal erythroderma (red baby). Indian J Paediatr Dermatol 2013;14:47-53.  Back to cited text no. 19
  [Full text]  
Vahlquist A, Blockhuys S, Steijlen P, van Rossem K, Didona B, Blanco D, et al. Oral liarozole in the treatment of patients with moderate/severe lamellar ichthyosis: Results of a randomized, double-blind, multinational, placebo-controlled phase II/III trial. Br J Dermatol 2014;170:173-81.  Back to cited text no. 20
Bassotti A, Moreno S, Criado E. Successful treatment with topical N-acetylcysteine in urea in five children with congenital lamellar ichthyosis. Pediatr Dermatol 2011;28:451-5.  Back to cited text no. 21
Hernández-Martín A, González-Sarmiento R. Recent advances in congenital ichthyoses. Curr Opin Pediatr 2015;27:473-9.  Back to cited text no. 22
Fortina AB, Bardazzi F, Berti S, Carnevale C, Di Lernia V, El Hachem M, et al. Treatment of severe psoriasis in children: Recommendations of an Italian expert group. Eur J Pediatr 2017;176:1339-54.  Back to cited text no. 23


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Recent Advances
Article Figures
Article Tables

 Article Access Statistics
    PDF Downloaded222    
    Comments [Add]    

Recommend this journal