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REVIEW ARTICLE
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
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_93_19

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  Abstract 


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 Jan 25];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)

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

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

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  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

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  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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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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
    
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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
    
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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
    
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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
    
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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
    
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Hernández-Martín A, González-Sarmiento R. Recent advances in congenital ichthyoses. Curr Opin Pediatr 2015;27:473-9.  Back to cited text no. 22
    
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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
    


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