Kyrle’s disease, first described in 1916, was known as hyperkeratosis follicularis et parafollicularis in cutem penetrans. Today, it is considered one of many acquired perforating dermatoses, which are often seen in uncontrolled diabetic or chronic renal failure patients. The lesions are characterized by large umbilicated papules and nodules with a central keratotic core. It is seen in both men and women equally, and usually occurs at the age of 30.
The cause remains unclear, yet is most likely thought to be caused by the elimination of keratin and cellular material at the outer skin layer. Some believe that this caused by an inflammatory response, while others believe it is a genetic inheritance.
The skin lesions are tender to touch and itchy. The lesions are described as red-brown papules or nodules with a central keratin plug. The lesions typically appear on the lower extremities, but may also appear on the arms and trunk. One case has appeared as an ocular lesion. Under a microscope, a parakeratotic plug is seen in the epidermis. Keratin material typically fills from the basal layer to the dermis, where broken down cellular debris and connective tissue go through transepidermal elimination.
Treatment is difficult and often not satisfying to the patient. Efforts should be directed at correcting the underlying conditions of diabetes and renal failure with strict compliance to dialysis and good blood sugar control. There are case reports of clinical cure occurring after renal transplant. Local wound care measures such as topical antibiotics or petrolatum should be applied to open lesions. Topical corticosteroids can provide some relief, and both systemic and topical retinoids have been effective for some patients. UV light therapy is also considered an effective approach due to the widespread and pruritic nature of the lesions.
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