Lamellar Ichthyosis

Key Points
Rare genetic disorder affecting newborns that unfortunately persists throughout life
Consists of thick, rough scales which covers the entire body

Lamellar Ichthyosis is a rare skin condition which affects newborn infants. At birth, the infant is often covered with a collodion membrane, which is a thick, membrane-like covering that usually desquamates or peels away after several days. Eventually it develops into a chronic pattern of large polygonal scales that cover the entire body. The scales are often attached centrally and raised at the edges. There is typically accentuation in the creases of the skin such as the anticubital and popliteal fossae. This may be accompanied by ectropion of the lower eyelids. Additionally, affected persons may experience susceptibility to overheating, limitation of movement, secondary skin infections, and even impaired vision or hearing later in life.

Lamellar Ichthyosis is a rare variant of Ichthyosis, in which a genetic mutation causes abnormal skin function, specifically increasing the rate at which new skin is formed or decreasing the rate at which it sheds, leading to the appearance of cutaneous symptoms. Lamellar Ichthyosis may affect anyone regardless of gender, age or ethnic group.

Differential Diagnosis (Other conditions with similar appearance)
Ichthyosis Fetalis
Ichthyosis Vulgaris, Hereditary and Acquired
Ichthyosis, X-Linked
Rud Syndrome
Sjogren-Larsson Syndrome

Diagnosis
Key Points
Diagnosis based on appearance of symptoms in the affected person

Treatment
No cure for the condition exists
Goal of treatment is to control symptoms and prevent secondary infection
Self-care is imperative in preventing complications
OTC Options: Heavy moisturizers, emollient creams containing urea, lactic or alpha hydroxy acids

There is no cure for lamellar Ichthyosis. The goal of treatment is to control the symptoms of the condition and to prevent secondary infection from occurring. Self-care measures, including daily bathing, exfoliation of skin, and moisturizing, are imperative in keeping the condition under control and preventing complications, as it prevents build up of skin cells and the attendant symptoms. Severe cases may call for health care professionals to administer more aggressive treatments, such as oral retinoids (acitretin, isotretinoin). Antibiotics may be prescribed if secondary infection is present.