Kyrle s disease is a rare dermatologic entity most commonly seen in patients receiving longterm hemodialysis for endstage renal disease. Convert html to pdf online, webhtml to pdf api pdfcrowd. Generally, kyrle s disease appears between 30 to 50 years of age and women are affected more than men. A case of infantile kyrleflegel disease in a 6yearold yemeni girl. Kyrles disease in a patient of diabetes mellitus and. The papules contain a central keratotic plug, which histologically correlates with keratin and necrotic debris.
Kyrle disease is a perforating skin condition characterized by the. We report a case of a 40yearold man, a worker in a cement factory, who noticed the first. Kyrle disease genetic and rare diseases information. The deep penetration of the keratin plugs, the size and irregularity of the papules, the age of onset and the location of the skin lesions can help differentiate kyrle disease from other conditions that are associated with similar skin findings. Recurrent venous thromboembolism in men and women nejm. June 17 issue1 found that the risk of recurrent venous thromboembolism is higher among men than among women. Acquired perforating dermatosis in patients with chronic renal failure. It is commonly seen in patients with diabetes, renal disease and rarely in liver disease. Kyrle s disease kd is a rare skin disorder characterized by transepidermal elimination of abnormal keratin. This finding could influence the management of secondary p.
Recently, however, there is a controversy on categorizing kyrle disease with perforating dermatosis or a subtype of acquired perforating collagenosis kyrle disease was first described by josef kyrle in 1916 when a. Kyrle s disease kd is an acquired perforating dermatosis associated with an underlying disorder such as diabetes mellitus or chronic renal failure. He had no known comorbidities such as diabetes mellitus and hypertension, or any other chronic kidney or liver disease. Biopsy of the skin lesion shows incomplete keratinization and the formation of a hyperkeratotic, parakeratotic plug. A variety of topical and systemic treatment options are available for the treatment. Kyrles disease in a patient of diabetes mellitus and chronic renal. Kyrle disease is a perforating skin condition characterized by the presence of large keratotic papules distributed widely throughout the body. 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. A clinicopathologic study of thirty cases of acquired perforating dermatosis in korea article pdf available in annals of dermatology 262.
A diagnosis of kyrle disease is often suspected based on the presence of characteristic signs and symptoms on physical examination. Pdf kyrles disease in a patient with delusions of parasitosis. Efficacy of topical tacrolimus for treating kyrles disease kinoshita. Kyrle disease is identified as a form of an acquired perforating disease. Kyrles disease kd is an acquired perforating dermatosis associated with an underlying disorder such as diabetes mellitus or chronic renal failure. Other major perforating diseases are elastosis perforans serpiginosa and reactive perforating collagenosis. Hyperkeratosis follicularis et parafollicularis in cutem. In their study of plasma levels of factor viii in relation to recurrent venous thrombosis, kyrle et al. Kyrles disease definition of kyrles disease by the. To this day controversy remains about the classification of kyrle disease is it a distinct disease entity, part of the spectrum of acquired perforating dermatosis or a subtype. Here, we report the first case of kyrles disease that was successfully treated by topical. Histologic findings in five cases of kyrles disease are described. Pdf acquired perforating disorders are a group of uncommon skin conditions characterized by transepidermal extrusion of altered dermal material, most.
Kyrle disease associated with hidradenitis suppurativa. Kyrle disease kd and flegel disease fd are rare variants of primary perforating dermatoses, characterized by. Full text full text is available as a scanned copy of the original print version. Kyrles disease is a chronic, genetically determined, hyperkeratotic disorder occurring in the middle forties. Kyrle s disease kd is a cutaneous disease that develops in individuals with underlying systemic disease, particularly chronic renal failure and diabetes mellitus dm, and is associated with a high burden of disease linked to itch. Convert html to pdf online in the browser or in your php, python, ruby. Get a printable copy pdf file of the complete article 4k, or click on a. A case of kyrles disease with dermatoscopic findings ncbi. It is classified as a subtype of acquired perforating dermatosis along with reactive perforating collagenosis, elastosis perforans serpiginosa and perforating folliculitis. Kyrles disease is a rare perforative skin dermatosis.
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