Kyrle's disease
Kyrle's Disease Kyrle's disease, also known as Kyrle's hyperkeratosis, is a rare dermatological condition characterized by the development of large, keratotic papules, primarily on the extremities. It is considered a type of acquired perforating dermatosis.
Clinical Presentation[edit | edit source]
Patients with Kyrle's disease typically present with hyperkeratotic papules that can range from 1 to 10 mm in diameter. These lesions are often pruritic and can become secondarily infected due to scratching. The papules are usually found on the extensor surfaces of the arms and legs, but they can also appear on the trunk and face.
Pathophysiology[edit | edit source]
The exact pathophysiology of Kyrle's disease is not well understood. It is thought to involve abnormal keratinization and transepidermal elimination of altered dermal material. This process results in the characteristic keratotic papules seen in affected individuals.
Associated Conditions[edit | edit source]
Kyrle's disease is often associated with systemic conditions such as:
These associations suggest that metabolic or systemic factors may play a role in the development of the disease.
Diagnosis[edit | edit source]
Diagnosis of Kyrle's disease is primarily clinical, based on the appearance of the characteristic lesions. A skin biopsy can be performed to confirm the diagnosis, revealing hyperkeratosis, parakeratosis, and transepidermal elimination of keratin and other dermal material.
Treatment[edit | edit source]
Treatment options for Kyrle's disease are limited and primarily symptomatic. They may include:
- Topical keratolytics such as salicylic acid or urea to reduce hyperkeratosis
- Topical corticosteroids to reduce inflammation and pruritus
- Systemic retinoids in severe cases
Management of any underlying systemic conditions is also crucial in the treatment of Kyrle's disease.
Prognosis[edit | edit source]
The prognosis for Kyrle's disease varies depending on the underlying systemic condition. While the skin lesions themselves are not life-threatening, they can cause significant discomfort and cosmetic concerns for the patient.
See Also[edit | edit source]
External Links[edit | edit source]
- [DermNet NZ - Kyrle's Disease](https://dermnetnz.org/topics/kyrles-disease/)
- [Orphanet - Kyrle's Disease](https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=999)
NIH genetic and rare disease info[edit source]
Kyrle's disease is a rare disease.
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Contributors: Prab R. Tumpati, MD
