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Kyrle disease - Skin
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Kyrle disease - Skin

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Contributors: Rosemara Hughart MD, Jeffrey D. Bernhard MD, Paul Kelly MD, Lowell A. Goldsmith MD, MPH, Lynn McKinley-Grant MD, Aída Lugo-Somolinos MD
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Synopsis

Kyrle's disease, also known as hyperkeratosis follicularis et parafollicularis in cutem penetrans, is one of four classic perforating dermatoses that are now grouped under the umbrella term of "acquired perforating dermatosis;" this grouping also includes elastosis perforans serpiginosa, perforating folliculitis, and reactive perforating collagenosis. Acquired perforating dermatoses are often associated with diabetes mellitus or chronic renal disease; however, they may also occur in patients with other systemic diseases, in association with medications, and in patients without an underlying disease. 

The cause of Kyrle's disease is unknown, but the pathomechanisms are thought to be multifactorial, including mechanical injury (especially from scratching), abnormal metalloproteinase activity, and transforming growth factor (TGF)-beta3 overexpression. Associations with nephropathy secondary to diabetes and with congestive heart failure and liver disease have been proposed. Age of onset is generally between 20 and 63 with an average age of onset of 30 years old. There seems to be no predilection for sex or race/ethnicity. The disease is rare except when seen in association with renal failure and is not uncommon in hemodialysis patients who have pruritus. Kyrle's lesions are found in about 10% of patients on hemodialysis secondary to diabetic nephropathy. Other precipitating factors that have been postulated include infection, disorder of vitamin A metabolism, and genetics (it has been hypothesized that this is an autosomal recessive genodermatosis).

Kyrle's disease classically presents as multiple discrete hyperkeratotic follicular (predominantly) and parafollicular eruptive papules with central crust or hyperkeratotic plug. When central plugs are removed, a pit-like depression remains (Kyrle's phenomenon). Pruritus may be present. Koebner's phenomenon (lesions forming at sites of skin trauma) is not generally seen, in contrast to some of the other perforating disorders. The predominant material that is extruded via transepidermal elimination in Kyrle's disease is keratin; elastic fibers and collagen can be present as well but are more prominent in other perforating disorders.

Pediatric Patient Considerations:
Cases documented in children are very rare.

Codes

ICD10CM:
L87.0 – Keratosis follicularis et parafollicularis in cutem penetrans

SNOMEDCT:
34042008 – Hyperkeratosis follicularis in cutem penetrans

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Last Updated: 01/14/2013
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Kyrle disease - Skin
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Kyrle disease : Bilateral, Follicular configuration, Peritoneal dialysis, Pruritus, Scaly papules, Legs
Clinical image of Kyrle disease
A close-up of several hyperkeratotic, deep pink papules, some with a prominent whitish portion centrally.
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