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[Netherton syndrome--a rare form of inherited ichtyosis]
Riitta Marttila1, Mirja Tuomiranta
1Seinäjoen keskussairaala.
Abstract:
Netherton syndrome is a rare skin disease classified into ichtyoses. It has a recessive pattern of inheritance. It is associated with scaly erythrodermia, bamboo hair defect, immunological abnormalities of varying severity, IgE-mediated allergic reactions, infections and defective temperature regulation that often leads to retarded growth and development of a newborn. The phenotype of the disease varies from mild skin symptoms to lethal forms of the disease. We describe two Finnish families, whose children were diagnosed with this disease.
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