Kyrle disease

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Kyrle disease

Kyrle disease, also known as hyperkeratosis follicularis et parafollicularis in cutem penetrans, is a rare skin disorder characterized by the development of large keratotic papules, primarily on the extremities. It was first described by the Austrian dermatologist Josef Kyrle in 1916.

Clinical Presentation[edit | edit source]

Kyrle disease typically presents with hyperkeratotic papules that can range from 1 to 10 mm in diameter. These lesions are often found on the legs, arms, and trunk, and they may be associated with pruritus (itching). The papules are usually skin-colored or brown and have a central keratin plug.

Pathophysiology[edit | edit source]

The exact cause of Kyrle disease is not well understood. It is believed to be related to metabolic disorders such as diabetes mellitus and chronic renal failure. The disease is characterized by the abnormal proliferation of keratinocytes and the formation of keratin plugs that penetrate the epidermis and dermis.

Diagnosis[edit | edit source]

The diagnosis of Kyrle disease is primarily clinical, based on the characteristic appearance of the lesions. A skin biopsy can be performed to confirm the diagnosis, revealing hyperkeratosis, parakeratosis, and the presence of keratin plugs.

Treatment[edit | edit source]

There is no definitive cure for Kyrle disease. Treatment is mainly symptomatic and may include the use of topical keratolytics, such as salicylic acid and urea, to reduce the hyperkeratosis. In some cases, systemic retinoids may be prescribed to help control the proliferation of keratinocytes.

Prognosis[edit | edit source]

The prognosis for Kyrle disease varies. While the condition is chronic and can be persistent, it is generally not life-threatening. The severity of symptoms can fluctuate, and some patients may experience periods of remission.

Related Conditions[edit | edit source]

Kyrle disease is part of a group of disorders known as follicular keratoses, which also includes conditions such as keratosis pilaris and lichen spinulosus.

See Also[edit | edit source]

References[edit | edit source]

External Links[edit | edit source]

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Contributors: Prab R. Tumpati, MD