Kyrle Disease

At a glance

It is a skin disease usually associated with systemic disorders such as chronic renal failure, diabetes mellitus, hepatic abnormalities, and congestive heart failure. It is characterized by widespread development of large papules with central keratin plugs. The presence of hyperkeratotic and hyperpigmented papules along the Blaschko lines over the trunk and extremities has been reported. 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.

History

This medical condition was first described by Josef Kyrle in 1916 when a diabetic woman presented generalized hyperkeratotic nodules.

Incidence

Rare, except in the setting of diabetes mellitus, chronic renal failure, or hepatic anomalies of metabolism. The most commonly affected patients are diabetics undergoing hemodialysis for chronic renal failure.

Genetic inheritance

Most likely it is not a genetic disorder in most instances, but some reports have suggested an autosomal dominant transmission. Most often associated with systemic disorders such as diabetes mellitus, hepatic abnormalities (alcoholic cirrhosis), congestive heart failure, and renal disease.

Pathophysiology

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 reaction. The initiating step may be an alteration of dermal connective tissue, which then is recognized and eliminated by proliferative epidermis.

Diagnosis

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 plug.

Clinical aspects

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 with multiple, tiny, yellow-brown stromal corneal opacities.

Precautions before anesthesia

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 ...