Lamellar recessive ichthyosis
Overview
A very rare skin disorder characterized by severe hardening and thickening of skin on all parts of the body.
Symptoms
* Large dark skin scales * Outward turned eyelids * Outward turned lips * Red skin under scales * Thickening of skin on palms * Hardening of skin on palms * Thickening of skin on soles * Hardening of skin on soles * Impaired sweating
Diagnosis
Begin the history by asking how long the patient has had scaly skin and whether he has had it before. Where did it first appear? Did a lesion or skin eruption, such as erythema, precede it? Has the patient used a new or different topical skin product recently? How often does he bathe? Has he had recent joint pain, illness, or malaise? Ask the patient about work exposure to chemicals, use of prescribed drugs, and a family history of skin disorders. Find out what kinds of soap, cosmetics, skin lotion, and hair preparations he uses. Next, examine the entire skin surface. Is it dry, oily, moist, or greasy? Observe the general pattern of skin lesions, and record their location. Note their color, shape, and size. Are they thick or fine? Do they itch? Does the patient have other lesions besides scaly skin? Examine the mucous membranes of his mouth, lips, and nose, and inspect his ears, hair, and nails.
Treatment
Teach the patient proper skin care, and suggest lubricating baths and emollients. Instruct him not to use hot water to bathe or shower.