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 with asthma, eczema, and
Severe form of ichthyosis lamellaris in an infant.
Ichthyosis lamellaris in a teenager.
Autosomal dominant inheritance.
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.
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.
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.
Evaluate hydration status in case of
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
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.
Multiple forms of ichthyosis.
DiGiovanna JJ, Robinson-Bostom L: Ichthyosis: Etiology, diagnosis, and
management. Am J Clin Dermatol
Okulicz JF, Schwartz RA: Hereditary and acquired ichthyosis vulgaris. Int J Dermatol
Smart G, Bradshaw EG: Extradural analgesia and ichthyosis. Anaesthesia