ichthyosis lamellar type 1

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Pathology

Genetics

Clinical manifestations

  • one the most severe forms of ichthyoses apparent at birth & persisting throughout life
  • patients are born encased in a tight, shiny, translucent covering called collodion membrane
  • over the first weeks of life, the collodion membrane is gradually replaced by generalized large, dark brown, plate-like scales with minimal to no erythroderma
  • tautness of facial skin commonly results in ectropion, eclabium & scarring alopecia of the scalp

Complications

More general terms

References

Database