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Erythroderma desquamativum

Disease definition

A rare immune deficiency with skin involvement characterized by early infantile onset of a clinical tetrad comprising generalized severe seborrheic-like erythroderma, recurrent secondary bacterial or fungal infections (most commonly Staphylococcus aureus, Candida, and gram-negative bacteria), persistent, profuse malabsorptive diarrhea, and failure to thrive or marked wasting. Associated systemic symptoms include fever, anemia, and weight loss. Further critical complications are impaired thermoregulation and severe fluid loss due to extensive exfoliation.

ORPHA:314

Classification level: Disorder
  • Synonym(s):
    • Leiner disease
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: L21.1
  • ICD-11: EH40.2
  • OMIM: 609536
  • UMLS: -
  • MeSH: -
  • GARD: 6878
  • MedDRA: -

  A summary on this disease is available in Español (2021) Français (2021) Nederlands (2021)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

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