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Related to lamellar ichthyosis: congenital ichthyosiform erythroderma


A congenital skin disease characterized by dryness and scales, especially on the extensor surfaces of the extremities.



fish skin disease, xeroderma, a skin disease characterized by sharply increased keratogenesis and retarded keratolysis.

Incidence of ichthyosis is a familial condition in half the cases and hereditary in 25 percent. It appears in early childhood (most often around age three), intensifies at puberty, and lasts throughout life. It is expressed in dryness of the skin and the accumulation of horny masses on the skin’s surface, in the form of scales or of massive horny plates that resemble fish scales. The excretion of oil and perspiration decreases sharply. The affection spreads over the entire skin surface, except at joint folds, axillae, and inguinal folds. On the face and scalp, ichthyosis appears in the form of furfuraceous desquamation. The condition of the skin improves in the summertime as a result of increased excretion of oil and sweat. Ichthyosis is treated with hot baths containing soda or table salt, followed by lubrication of the skin with emollient creams. Cod-liver oil and vitamins may be taken internally (in particular vitamin A). In the summer, prolonged sojourns in the south can be helpful (sea bathing).


References in periodicals archive ?
On the basis of these findings, a diagnosis of lamellar ichthyosis with rickets was made and the child was treated with parenteral vitamin D3 (600,000 units, intramuscular monthly) and oral cal- cium (1000 mg daily in two divided doses) with vi- tamin D supplements.
2 Lamellar ichthyosis is an autosomal recessive disorder that is apparent at birth and persists throughout life.
Lamellar ichthyosis (LI) is relatively rare type of ichthyosis with an incidence of approximately 1:200,000 to 300,000 live births.
6 To date, 6 genes for lamellar ichthyosis have been localized and 5 of them identified, as follows2: TGM1 (14q11), ABCA12 (2q34), 19p12-q12, 19p13, ALOXE3- ALOX12B (17p13), ichthyin (5q33).