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Kyrle Disease

  • Author: Daniel J Hogan, MD; Chief Editor: William D James, MD  more...
 
Updated: Sep 18, 2014
 

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

United States

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]

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 College of Osteopathic Medicine; 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, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research, Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation

Disclosure: Received consulting fee from Valeant for consulting; Received grant/research funds from Medicis for other; Received consulting fee from Galderma for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Pharmaderm for consulting; Received consulting fee from Onset for consulting.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, James W. Patterson, MD, to the development and writing of this article.

References
  1. Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of literature. Arch Dermatol. 1968 Jun. 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. 1968 Jun. 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. 1989 Aug. 125(8):1074-8. [Medline].

  4. White CR Jr, Heskel NS, Pokorny DJ. Perforating folliculitis of hemodialysis. Am J Dermatopathol. 1982 Apr. 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. 1982 Apr. 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. 2000 Oct. 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. 2005 Jun. 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. 1985 Jun. 7(3):271-6. [Medline].

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

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

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

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

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

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A typical lesion of Kyrle disease with central keratotic crater.
 
 
 
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