Information on Kyrle Disease

Kyrle disease is characterized by the formation of large papules with central keratin plugs that may grow in a widespread distribution pattern. The illness is most closely connected with diabetes mellitus and renal failure. In the United States and internationally, Kyrle disease is rare, except in the setting of chronic renal failure. With chronic renal failure, perforating dermatoses are more common. It can influence both men and women throughout life, although the average age at time of presentation is 30 years.

The cause of the disease is idiopathic, or inherited. Kyrle disease appears to arise more often in patients with certain systemic disorders, such as, diabetes mellitus, renal disease (chronic renal failure, albuminuria, prominent serum creatinine, abnormal creatinine clearance, and polyuria), hepatic abnormalities (alcoholic cirrhosis) and congestive heart failure. Its occurrence in a familial setting, especially in children, is very uncommon. Similar appearing skin lesions have been described in adults. Lesions begin as small papules with silvery scales that eventually grow to about 1.5cm in diameter to form red-brown nodules with a central keratin plug.

Multiple lesions may combine to form large keratotic plaques. Lesions happen generally on the legs but also grow on the arms and in the head and neck region. The palms and soles are infrequently affected.  Lesions are not painful but patients may experience intense pruritus (itching). Lesions may self-heal without any treatment but often new lesions develop. Treatments that have been used to treat and decrease lesions include isotretinoin, high dose vitamin A and tretinoin cream. Another approach is administration of oral retinoid. For patients in whom itching is a major problem, soothing antipruritic lotions may be helpful.