Kyrle Disease

Updated: Apr 18, 2017
  • Author: Mary Piazza Maiberger, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Kyrle disease is a perforating skin condition characterized by the presence of large keratotic papules distributed widely throughout the body. The papules contain a central keratotic plug, which histologically correlates with keratin and necrotic debris. The disease is most closely associated with diabetes mellitus and renal failure.

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Pathophysiology

The pathophysiology of Kyrle disease is unclear. Some believe it is a variant of prurigo nodularis or may represent end-stage excoriations of a folliculitis on the legs in patients with renal failure.

The leading theory is that the disease represents transepidermal elimination of keratin and other cellular material. Carter and Constantine [1, 2] have suggested that in Kyrle disease keratinization focally occurs at the basilar layer of the epidermal, rather than normal proliferation with keratinization higher in the epidermis. This elicits a host inflammatory response, resulting in keratin, cellular material, and connective tissue being forced out of the skin through the epidermis. [3] Alteration of dermal connective tissue may also be an initiating step, causing an inflammatory response. Other skin diseases with altered connective tissue have a similar type of inflammatory response, such as elastosis perforans serpiginosa, and perforating collagenosis.

Some literature suggests that Kyrle disease may be a recessively inherited genodermatosis.

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Epidemiology

Frequency

In the United States and internationally, Kyrle disease is rare, except in the setting of chronic renal failure. In patients with chronic renal failure, perforating dermatoses (that are closely related to and probably represent variants of Kyrle disease) are more common. Kyrle Disease occurs in 10% of dialysis patients. [4, 5]

Race

Kyrle disease appears to be more common in African Americans, perhaps related to the high incidence of diabetes mellitus and renal failure in this population.

Sex

This disorder may be more common in women.

Age

A wide age range exists among patients with Kyrle disease. The average age at time of presentation is 30 years.

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Prognosis

Improvement of skin lesions is possible. The ultimate prognosis depends upon the nature of any underlying systemic disease. Morbidity results from the appearance of the lesions and the intense itching that often is associated with the condition. However, significant morbidity and mortality may be more directly associated with the underlying disease (eg, diabetes mellitus, chronic renal failure, hepatic abnormalities).

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