Netherton Syndrome

Netherton Syndrome is a rare syndrome characterized by red, scaly skin, short brittle hair and a predisposition to allergies, asthma and eczema. Newborns with Netherton syndrome have reddened skin (erythroderma) and, occasionally, a thick shell-like covering of skin (collodion membrane). They may also be born prematurely. Trouble gaining weight during infancy and childhood is common. Atopic dermatitis (red, itchy patches of skin) may be present, and a cradle cap-like scale and redness may appear on the face, scalp and eyebrows.

Unlike many of the ichthyoses, Netherton syndrome produces too few layers of the outer skin, instead of two many layers. Therefore, agents that remove scale, such as the alpha-hydroxy acids and oral retinoids, are not helpful in the management of this disorder and may aggravate the symptoms. Current treatment options are limited to use of mild moisturizers containing petrolatum or lanolin and/or a barrier repair formula containing ceramides or cholesterol.