Keratosis follicularis spinulosa decalvans
Overview
A rare inherited skin disorder affecting mostly males and characterized by hardening of skin around hair follicles which leads to scarring and loss of hair. The skin on the face, neck, forearms and other areas can also be affected
Symptoms
* Thickening of follicular skin * Patchy hair loss * Progressive scarring * Itching * Progressive baldness * Sparse eyelashes * Sparse eyebrows * Sensitivity to light * Excess tearing * Corneal abnormalities * Inflammation of eyelids * Ectropion * Telangiectasia in cheeks * Underdeveloped lower jaw bone * Follicular ichthyosis * Corneal lesions
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.