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CDSN-nEDD

Previously: Peeling skin syndrome

nEDD Gene: CDSN AR

Onset: Birth

Pathway: Structural proteins

Category Paper: nEDD Paper

CDSN-nEDD: diffuse erythema and superficial peeling of the skin leaving patchy areas of more pronounced erythema.

Key Clinical Clues

Generalized superficial skin peeling with erythema, intractable pruritus, atopic diathesis.

Additional Cutaneous Features

Histopathology: If biopsied in the centre of the lesion: superficial detachment of the horny layer.

References

Oji V, Eckl K-M, Aufenvenne K et al. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unravelling the peeling skin disease. Am J Hum Genet 2010; 87:274-81.

Summarised from published literature for general information. This is not medical advice, and entries are still being verified. Read the full disclaimer