Erythroderma desquamativum
Synonyms: Leiner disease
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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Reference: Access aggregated data from Orphanet at Orphadata.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024
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Erythroderma desquamativum?
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