Skin disease usually associated with systemic
disorders such as chronic renal failure, diabetes mellitus, hepatic
abnormalities, and congestive heart failure. Characterized by widespread
development of large papules with central keratin plugs. Intense itching is
often present. Skin lesions are present for 4 months to 4 years, but most
often respond to the treatment of the underlying cause. Other features
include coagulation disorders, albuminemia, elevated serum creatinine, and
polyuria. Onset age is approximately 30 years.
Rare, except in the setting of diabetes mellitus, chronic renal failure, or hepatic anomalies
of metabolism. The most commonly affected patients are diabetics undergoing
hemodiolysis for chronic renal failure.
Most likely not a genetic disorder in most
instances, but some reports suggest an autosomal dominant transmission.
Most often associated with systemic disorders such as diabetes mellitus,
hepatic abnormalities (alcoholic cirrhosis), congestive heart failure, and
Not clearly established. Characterized by multiple
hyperkeratotic papules with penetration of a keratotic plug into the dermis,
resulting in transepidermal elimination
of keratin, cellular material, and connective tissue elements triggered by an inflammatory
initiating step may be an alteration of dermal connective tissue, which then
is recognized and eliminated by proliferative epidermis.
Histopathologic examination shows a central, thick hyperkeratotic and parakeratotic
plug embedded in an epidermal invagination. The plug consists of basophilic material.
Abnormal (parakeratotic) keratinization of all the epithelial cells,
including the basal cells, is present in at least one deep region of the
The average age at presentation is 30 years,
however, childhood cases have also been described.
Flesh-colored, hard, horny papules may develop anywhere on the body except
for palms and soles.
Most commonly the extensor surfaces of the extremities are affected.
Rarely, it may also involve the mucous membranes (conjunctivae, buccal mucosa).
The papules eventually acquire a
central keratotic plug that, upon removal, leaves a crater that matches the
shape of the plug (Kyrle sign). The lesions come in crops, last for several
weeks, and eventually disappear with minimal or no scarring. The lesions are
asymptomatic. Posterior subcapsular cataracts may occur, as may multiple,
tiny, yellow-brown stromal corneal opacities.
Hepatic functions must be evaluated
for coagulation disorders and hypoalbuminemia. Glucose
homeostasis should be established prior to anesthesia. Creatinine level and
clearance must be assessed.
A complete blood count is recommended (renal anemia).
In presence of severe itching, skin infections
should be ruled out.
Difficult venous access
should be expected
because of the skin
condition. In presence of congestive heart failure, chronic renal failure,
and/or hepatic dysfunction, all considerations specific to these
underlying conditions apply (rather than to Kyrle disease itself).
Renal and/or hepatic insufficiency should be kept in mind when selecting drugs.
Hypoalbuminemia may alter protein-binding characteristics of drugs and reduced
drug doses may be required.
Alyahya GA, Heegaard S, Prause JU: Ocular changes in a case of Kyrle's
disease: 20-year follow-up. ...