Inherited as an autosomal recessive trait, lamellar ichthyosis manifests as retained keratin in large brown scales that lift at the periphery.
When the face is involved, ectropion may be evident.
Because of a hair shaft defect, hairs may be sparse or absent.
Extensive involvement of the elbows, knees and ankles, as well as involvement of the palms and soles is common.
Clinically, this condition can be distinguished from ichthyosis vulgaris by the larger, often brown scales found in lamellar ichthyosis.
- Alpha-hydroxy acids, such as tartaric acid 5% in an ointment base are often strikingly effective.
- Ammonium lactate 12% lotion is also helpful to some patients.
- Because of the extent of the skin involvement, there can be significant systemic absorption of salicylic acid, should that preparation be used.
- Oral retinoids such as etretinate may be of considerable benefit, but as treatment must be continued for life, treatment with retinoids should be started only with circumspection.