Netherton's Syndrome: A Syndrome of Elevated IgE and Characteristic Skin and Hair Findings
January 1995
in “
The journal of allergy and clinical immunology/Journal of allergy and clinical immunology/The journal of allergy and clinical immunology
”
Netherton's syndrome IgE atopic symptoms ichthyosis linearis circumflexa congenital lamellar ichthyosis trichorrhexis invaginata atopic dermatitis topical corticosteroids ammonium lactate lotion Netherton's syndrome IgE atopic symptoms ichthyosis linearis circumflexa congenital lamellar ichthyosis trichorrhexis invaginata atopic dermatitis topical corticosteroids ammonium lactate lotion
TLDR Netherton's syndrome is linked to high IgE levels and unique skin and hair symptoms, and may improve with ammonium lactate lotion and allergy management.
Netherton's syndrome (NS) was characterized by elevated IgE levels and distinct skin and hair abnormalities, including ichthyosis linearis circumflexa and trichorrhexis invaginata. A study of a 3-year-old patient showed significant improvement in skin condition with 12% ammonium lactate lotion and management of allergic symptoms. A review of 43 cases revealed common features such as TI (100%), ichthyosis linearis circumflexa (80%), and atopic manifestations. NS often presented with erythroderma, pruritus, and decreased sweating, with immunologic abnormalities including elevated IgE levels (982 to 15,302 IU/ml). Growth issues, mental retardation, and food allergies were also noted. Unlike atopic dermatitis, NS did not show white dermographism or thrombocytopenia. The underlying cause was suggested to involve a defect in a regulatory gene for IgE production or a defective intestinal mucosal barrier. Allergy evaluations and avoidance of allergens were recommended for managing atopic symptoms.
