Introduction
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.
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 Constantine1,2 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.
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.
Clinical
History
A history of an eruption of characteristic skin lesions exists, with duration ranging from 4 months to 43 years. The lesions may be asymptomatic, but tenderness and especially pruritus are reported. Without treatment, the eruption is persistent. However, the condition has been reported to clear following therapy of the underlying systemic disease (eg, diabetes mellitus, renal failure).
Physical
The primary lesion is a small papule with silvery scale. This eventually enlarges to form a red-brown papule or nodule with a central keratin plug (Media File 1). Some, but not all, of the lesions appear to be follicular. These may coalesce to form larger keratotic plaques. Koebnerization is not ordinarily described as such, but a striking linearity of lesions sometimes is noted. The papules, nodules, and plaques of Kyrle disease tend to occur primarily on the legs, but also develop on the arms and in the head and neck region. Involvement of palms and soles is rare. Ocular changes, including keratotic lesions of conjunctiva and cornea, have been described in a single case report.6
Causes
As indicated above, debate about the precise pathophysiology of the disease exists. Some cases appear to be idiopathic or inherited, but other examples of Kyrle disease are associated with systemic disorders. A case report by Kasiakou et al7 noted regression of lesions following antimicrobial therapy, suggesting a role for bacterial infection in pathogenesis. The following list includes several of the associated systemic disorders:
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| Differential Diagnoses & Workup: Kyrle Disease |
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References
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].
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].
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].
White CR Jr, Heskel NS, Pokorny DJ. Perforating folliculitis of hemodialysis. Am J Dermatopathol. Apr 1982;4(2):109-16. [Medline].
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].
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].
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].
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].
Salomon RJ, Baden TJ, Gammon WR. Kyrle's disease and hepatic insufficiency. Arch Dermatol. Jan 1986;122(1):18-9. [Medline].
Saleh HA, Lloyd KM, Fatteh S. Kyrle's disease. Effectively treated with isotretinoin. J Fla Med Assoc. Jun 1993;80(6):395-7. [Medline].
Bolognia JL, Rapini RP, Jorizzo JL, eds. Perforating Diseases. In: Dermatology. Vol 2. 2nd ed. St. Louis, Mo: Mosby; 2008:Chapter 95.
Cunningham SR, Walsh M, Matthews R, Fulton R, Burrows D. Kyrle's disease. J Am Acad Dermatol. Jan 1987;16(1 Pt 1):117-23. [Medline].
Ferdinand KC. Cardiovascular disease in blacks: can we stop the clock?. J Clin Hypertens (Greenwich). May 2008;10(5):382-9. [Medline].
Golusin Z, Poljacki M, Matovic L, Tasic S, Vuckovic N. [Kyrle's disease]. Med Pregl. Jan-Feb 2002;55(1-2):47-50. [Medline].
Patterson JW. The perforating disorders. J Am Acad Dermatol. Apr 1984;10(4):561-81. [Medline].
Price ML, Jones EW, MacDonald DM. Flegel's disease, not Kyrle's disease. J Am Acad Dermatol. Jun 1988;18(6):1366-7. [Medline].
Schamroth JM, Kellen P, Grieve TP. Atypical Kyrle's disease. Int J Dermatol. Jun 1986;25(5):310-3. [Medline].
Tappeiner J, Wolff K, Schreiner E. [Kyrle's disease]. Hautarzt. Jan 1969;20(1):296-310. [Medline].
Tardio ML, Fasano D, Marucci G, Collina G. [Hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle's disease). Description of a case]. Pathologica. Jun 2000;92(3):195-7. [Medline].
Further Reading
Keywords
Kyrle disease, Kyrle's disease, hyperkeratosis follicularis et parafollicularis in cutem penetrans, acquired perforating dermatosis, acquired reactive perforating collagenosis, acquired reactive perforating dermatosis, perforating disorder of uremia
