Kyrle disease

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

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

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

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

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

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

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

• Keratosis pilaris
• Lichen spinulosus
• Diabetes mellitus
• Chronic renal failure
• Hyperkeratosis