Skip to Main Content

At a glance

Group of cutaneous disorders of keratinization representing more than 95% of ichthyosis cases. Can be a genetic disorder that is generally present soon after birth, or it can be a nonhereditary condition associated with internal disease, such as malignancies, or it can be induced by various medications. Most patients present medical conditions such as asthma, eczema, and fever.

Synonyms

Ichthyosis Lamellaris Syndrome; Ichthyosis Simplex; Autosomal Dominant Ichthyosis.

Incidence

It is the most common form of ichthyosis as it affects 1:250 people in the United States. Males and females are affected equally.

Genetic inheritance

Autosomal dominant inheritance. However, there is a very rare acquired form described.

Pathophysiology

Hereditary forms can be caused by altered profilaggrin expression leading to scaling and desquamation and is often associated with atopy. It is a retention hyperkeratosis; expression of profilaggrin is absent or reduced in the epidermis.

Diagnosis

At birth, skin is normal. It gradually becomes rough and dry in early childhood. Scaling tends to be most prominent on the extensor surfaces of the extremities and absent on the flexor surfaces. Lesions are rare in the axillae and antecubital and popliteal fossae. Forehead and cheeks may be involved, but lesions in these areas diminish with age. Notable amelioration in summer.

Clinical aspects

Features involve mainly skin with scales (fine, irregular, and polygonal in shape) of various sizes and colors. Lower extremities generally are more affected than the upper extremities. The face is generally spared; however, hyperkeratosis of the palms and soles and follicular hyperkeratosis of the cheek and neck is often present. Pruritus is frequent. Fissures can appear with secondary infections. Inflammation is possible.

Precautions before anesthesia

Evaluate hydration status in case of extensive ichthyosis.

Anesthetic considerations

Fixation of monitoring devices and indwelling catheters on skin can be difficult. There is an increased risk of superinfection following installation of venous puncture, regional anesthesia, and any other maneuvers leading to skin damage. Patients affected are susceptible to thermoregulatory imbalance leading rapidly to severe hypothermia.

Pharmacological implications

Nicotinic acid, triparanol, butyrophenones, dixyrazine, cimetidine, and clofazimine have been implicated in acquired ichthyosis and should be avoided. Consider the administration of steroid stress doses in case of long-term treatment.

Other conditions to be considered

Multiple forms of ichthyosis.

Ichthyosis Lamellaris Syndrome: Ichthyosis lamellaris in a teenager.

Overview: “Ichthyoses” or “disorders of cornification” are general terms describing a group of scaly skin disorders. They are characterized by an abnormal accumulation squames in the ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.