Kyrle Disease 

  • Author: Daniel J Hogan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 13, 2010
 

Background

Kyrle disease is characterized by the formation of large papules with central keratin plugs that may develop in a widespread distribution pattern. Histopathologically, a keratin plug mingled with necrotic cellular debris and degenerated connective tissue occupies an epidermal invagination. The disease is most closely associated with diabetes mellitus and renal failure.

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Pathophysiology

Pathophysiology is unknown. Some believe Kyrle disease is a variant of prurigo nodularis on the legs in patients with renal failure or that it merely represents the presence of end-stage excoriated hyperplastic nodules of folliculitis.

The concept of an extrinsic keratin plug penetrating the epidermis generally has been discredited. Carter and Constantine[1, 2] have suggested that, within the epidermis, keratinization focally occurs at the expense of proliferation, with keratinization eventually occurring at the level of the basilar layer. This elicits a host inflammatory response, resulting in transepidermal elimination of keratin, cellular material, and connective tissue elements.[3] It also is possible that the initiating step is alteration of dermal connective tissue, which then is recognized, engulfed, and eliminated by proliferative epidermis. Such a concept is supported by similar processes that occur in conditions of altered connective tissue, such as elastosis perforans serpiginosa, perforating pseudoxanthoma elasticum, and reactive perforating collagenosis.

Experimental evidence has also shown that foreign particles, when located in the superficial dermis, undergo transepidermal elimination. Factors that might contribute to such a process include metabolic derangements, infection, mechanical trauma (eg, rubbing, scratching), or the springlike mechanism created by coiled-up hairs within hyperkeratotic follicular lumina. Some literature suggests that Kyrle disease may be a recessively inherited genodermatosis.

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Epidemiology

Frequency

United States

In the United States and internationally, Kyrle disease is rare, except in the setting of chronic renal failure. 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]

Mortality/Morbidity

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).

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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Contributor Information and Disclosures
Author

Daniel J Hogan, MD  Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center

Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Marjan Garmyn, MD, PhD  Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of literature. Arch Dermatol. Jun 1968;97(6):624-32. [Medline].

  2. Constantine VS, Carter VH. Kyrle's disease. II. Histopathologic findings in five cases and review of the literature. Arch Dermatol. Jun 1968;97(6):633-9. [Medline].

  3. Rapini RP, Herbert AA, Drucker CR. Acquired perforating dermatosis. Evidence for combined transepidermal elimination of both collagen and elastic fibers. Arch Dermatol. Aug 1989;125(8):1074-8. [Medline].

  4. White CR Jr, Heskel NS, Pokorny DJ. Perforating folliculitis of hemodialysis. Am J Dermatopathol. Apr 1982;4(2):109-16. [Medline].

  5. Hurwitz RM, Melton ME, Creech FT 3rd, Weiss J, Handt A. Perforating folliculitis in association with hemodialysis. Am J Dermatopathol. Apr 1982;4(2):101-8. [Medline].

  6. Alyahya GA, Heegaard S, Prause JU. Ocular changes in a case of Kyrle's disease. 20-year follow-up. Acta Ophthalmol Scand. Oct 2000;78(5):585-9. [Medline].

  7. Kasiakou SK, Peppas G, Kapaskelis AM, Falagas ME. Regression of skin lesions of Kyrle's disease with clindamycin: implications for an infectious component in the etiology of the disease. J Infect. Jun 2005;50(5):412-6. [Medline].

  8. Kahana M, Trau H, Dolev E, Schewach-Millet M, Gilon E. Perforating folliculitis in association with primary sclerosing cholangitis. Am J Dermatopathol. Jun 1985;7(3):271-6. [Medline].

  9. Salomon RJ, Baden TJ, Gammon WR. Kyrle's disease and hepatic insufficiency. Arch Dermatol. Jan 1986;122(1):18-9. [Medline].

  10. Khandpur S, Bansal A, Ramam M, et al. Verrucous tuberculid mimicking Kyrle disease. Int J Dermatol. Dec 2007;46(12):1298-301. [Medline].

  11. Saleh HA, Lloyd KM, Fatteh S. Kyrle's disease. Effectively treated with isotretinoin. J Fla Med Assoc. Jun 1993;80(6):395-7. [Medline].

  12. Bolognia JL, Rapini RP, Jorizzo JL, eds. Perforating Diseases. In: Dermatology. Vol 2. 2nd ed. St. Louis, Mo: Mosby; 2008:Chapter 95.

  13. Price ML, Jones EW, MacDonald DM. Flegel's disease, not Kyrle's disease. J Am Acad Dermatol. Jun 1988;18(6):1366-7. [Medline].

 
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A typical lesion of Kyrle disease with central keratotic crater.
 
 
 
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