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Neonatal and infantile erythroderma

Moise L Levy, MD
Section Editor
Jennifer L Hand, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Erythroderma is a generalized and persistent erythema of the skin involving at least 90 percent of the body surface [1]. Although relatively uncommon in infants, erythroderma may be the presenting feature of a wide range of acquired and inherited diseases, including infections, inflammatory skin diseases, ichthyoses, and congenital immunodeficiencies.

This topic will review the causes of erythroderma in neonates and infants and the approach to the diagnosis and management. Erythroderma in adults is discussed separately. (See "Erythroderma in adults".)


Neonatal and infantile erythrodermas are associated with a wide range of cutaneous and systemic disorders, including [1-3]:

Infectious diseases (eg, staphylococcal scalded skin syndrome, congenital cutaneous candidiasis, syphilis)

Inflammatory diseases (eg, atopic dermatitis, seborrheic dermatitis, psoriasis)

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Literature review current through: Oct 2017. | This topic last updated: Jul 02, 2017.
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  1. Levy ML. Erythrodermas, immunodeficiency, and metabolic disorders. In: Neonatal and Infant Dermatology, 3rd ed, Eichenfield LF, Frieden IJ, Mathes EF, Zaenglein AL (Eds), Elsevier, London 2014. p.265.
  2. Fraitag S, Bodemer C. Neonatal erythroderma. Curr Opin Pediatr 2010; 22:438.
  3. Ott H, Hütten M, Baron JM, et al. Neonatal and infantile erythrodermas. J Dtsch Dermatol Ges 2008; 6:1070.
  4. Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. J Eur Acad Dermatol Venereol 2014; 28:1418.
  5. Rostad CA, Philipsborn RP, Berkowitz FE. Evidence of staphylococcal toxic shock syndrome caused by MRSA in a mother-newborn pair. Pediatr Infect Dis J 2015; 34:450.
  6. Takahashi N, Uehara R, Nishida H, et al. Clinical features of neonatal toxic shock syndrome-like exanthematous disease emerging in Japan. J Infect 2009; 59:194.
  7. Takahashi N, Nishida H, Kato H, et al. Exanthematous disease induced by toxic shock syndrome toxin 1 in the early neonatal period. Lancet 1998; 351:1614.
  8. Takahashi N, Imanishi K, Uchiyama T. Overall picture of an emerging neonatal infectious disease induced by a superantigenic exotoxin mainly produced by methicillin-resistant Staphylococcus aureus. Microbiol Immunol 2013; 57:737.
  9. Darmstadt GL, Dinulos JG, Miller Z. Congenital cutaneous candidiasis: clinical presentation, pathogenesis, and management guidelines. Pediatrics 2000; 105:438.
  10. Lanternier F, Cypowyj S, Picard C, et al. Primary immunodeficiencies underlying fungal infections. Curr Opin Pediatr 2013; 25:736.
  11. Antaya RJ, Robinson DM. Blisters and pustules in the newborn. Pediatr Ann 2010; 39:635.
  12. Zhou Q, Wang L, Chen C, et al. A case series of 130 neonates with congenital syphilis: preterm neonates had more clinical evidences of infection than term neonates. Neonatology 2012; 102:152.
  13. Workowski KA, Bolan GA, Centers for Disease Control and Prevention. Sexually transmitted diseases treatment guidelines, 2015. MMWR Recomm Rep 2015; 64:1.
  14. Marquez L, Levy ML, Munoz FM, Palazzi DL. A report of three cases and review of intrauterine herpes simplex virus infection. Pediatr Infect Dis J 2011; 30:153.
  15. Lee R, Schwartz RA. Human T-lymphotrophic virus type 1-associated infective dermatitis: a comprehensive review. J Am Acad Dermatol 2011; 64:152.
  16. Pérez C L, Villarroel B J, Reyes J A, et al. [Exfoliative erythroderma and infective dermatitis in an infant infected with human T-lymphotropic virus type I (HTLV I)]. Rev Chilena Infectol 2007; 24:142.
  17. Takahashi H, Takahashi I, Tsuji H, et al. Analysis of psoriatic patients registered in Asahikawa Medical College Hospital from 1983 to 2007. J Dermatol 2009; 36:632.
  18. Kumar B, Jain R, Sandhu K, et al. Epidemiology of childhood psoriasis: a study of 419 patients from northern India. Int J Dermatol 2004; 43:654.
  19. Bender AM, Levy ML. The child with recalcitrant dermatitis: when to worry? Dermatol Clin 2013; 31:223.
  20. Aksentijevich I, Masters SL, Ferguson PJ, et al. An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist. N Engl J Med 2009; 360:2426.
  21. Marrakchi S, Guigue P, Renshaw BR, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620.
  22. Chang SE, Choi JH, Koh JK. Congenital erythrodermic psoriasis. Br J Dermatol 1999; 140:538.
  23. Salleras M, Sanchez-Regaña M, Umbert P. Congenital erythrodermic psoriasis: case report and literature review. Pediatr Dermatol 1995; 12:231.
  24. Leclerc-Mercier S, Bodemer C, Bourdon-Lanoy E, et al. Early skin biopsy is helpful for the diagnosis and management of neonatal and infantile erythrodermas. J Cutan Pathol 2010; 37:249.
  25. Koga H, Kokubo T, Akaishi M, et al. Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature. Pediatr Dermatol 2011; 28:542.
  26. Lange M, Niedoszytko M, Renke J, et al. Clinical aspects of paediatric mastocytosis: a review of 101 cases. J Eur Acad Dermatol Venereol 2013; 27:97.
  27. Lange M, Niedoszytko M, Nedoszytko B, et al. Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature. J Eur Acad Dermatol Venereol 2012; 26:1565.
  28. Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: practical recommendations. Am J Clin Dermatol 2011; 12:259.
  29. Alvarez-Twose I, Vañó-Galván S, Sánchez-Muñoz L, et al. Increased serum baseline tryptase levels and extensive skin involvement are predictors for the severity of mast cell activation episodes in children with mastocytosis. Allergy 2012; 67:813.
  30. Oji V, Tadini G, Akiyama M, et al. Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sorèze 2009. J Am Acad Dermatol 2010; 63:607.
  31. Auada MP, Taube MB, Collares EF, et al. Sjögren-Larsson syndrome: biochemical defects and follow up in three cases. Eur J Dermatol 2002; 12:263.
  32. Pruszkowski A, Bodemer C, Fraitag S, et al. Neonatal and infantile erythrodermas: a retrospective study of 51 patients. Arch Dermatol 2000; 136:875.
  33. Al-Herz W, Nanda A. Skin manifestations in primary immunodeficient children. Pediatr Dermatol 2011; 28:494.
  34. Lehman H. Skin manifestations of primary immune deficiency. Clin Rev Allergy Immunol 2014; 46:112.
  35. Abrams M, Paller A. Genetic immunodeficiency diseases. Adv Dermatol 2007; 23:197.
  36. Notarangelo LD. Primary immunodeficiencies. J Allergy Clin Immunol 2010; 125:S182.
  37. Puck JM. The case for newborn screening for severe combined immunodeficiency and related disorders. Ann N Y Acad Sci 2011; 1246:108.
  38. Freeman AF, Holland SM. The hyper-IgE syndromes. Immunol Allergy Clin North Am 2008; 28:277.
  39. Freeman AF, Holland SM. Clinical manifestations of hyper IgE syndromes. Dis Markers 2010; 29:123.
  40. Engelhardt KR, McGhee S, Winkler S, et al. Large deletions and point mutations involving the dedicator of cytokinesis 8 (DOCK8) in the autosomal-recessive form of hyper-IgE syndrome. J Allergy Clin Immunol 2009; 124:1289.
  41. Chu EY, Freeman AF, Jing H, et al. Cutaneous manifestations of DOCK8 deficiency syndrome. Arch Dermatol 2012; 148:79.
  42. Yu S, Graf WD, Shprintzen RJ. Genomic disorders on chromosome 22. Curr Opin Pediatr 2012; 24:665.
  43. Bedocs LA, O'Regan GM, Bruckner Al. Red, Scaly Babies: Neonatal Erythroderma. NeoReviews 2011; 12:e325.
  44. D'hauw A, Seyger MM, Groenen PJ, et al. Cutaneous graft-versus-host-like histology in childhood. Importance of clonality analysis in differential diagnosis. A case report. Br J Dermatol 2008; 158:1153.
  45. Katugampola RP, Morgan G, Khetan R, et al. Omenn's syndrome: lessons from a red baby. Clin Exp Dermatol 2008; 33:425.
  46. Baker MW, Grossman WJ, Laessig RH, et al. Development of a routine newborn screening protocol for severe combined immunodeficiency. J Allergy Clin Immunol 2009; 124:522.
  47. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol 2002; 47:386.
  48. Edwards AM, Capková S. Oral and topical sodium cromoglicate in the treatment of diffuse cutaneous mastocytosis in an infant. BMJ Case Rep 2011; 2011.
  49. Craiglow BG. Ichthyosis in the newborn. Semin Perinatol 2013; 37:26.
  50. Digiovanna JJ, Mauro T, Milstone LM, et al. Systemic retinoids in the management of ichthyoses and related skin types. Dermatol Ther 2013; 26:26.
  51. Al-Dhalimi MA. Neonatal and infantile erythroderma: a clinical and follow-up study of 42 cases. J Dermatol 2007; 34:302.
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