Hyperkeratosis follicularis et parafollicularis in cutem penetrans
=Hyperkeratosis Follicularis et Parafollicularis in Cutem Penetrans =
Hyperkeratosis Follicularis et Parafollicularis in Cutem Penetrans, also known as Kyrle's disease, is a rare dermatological condition characterized by the development of hyperkeratotic papules. These papules are typically found on the extensor surfaces of the limbs and can be associated with systemic diseases such as diabetes mellitus and renal failure.
Clinical Presentation[edit | edit source]
Patients with Kyrle's disease present with hyperkeratotic papules that are often pruritic. These lesions are typically 1-3 mm in diameter and have a central keratotic plug. The papules are most commonly found on the extensor surfaces of the arms and legs, but 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 believed to involve abnormal keratinization and follicular plugging. The condition is often associated with systemic diseases such as diabetes mellitus, chronic renal failure, and liver disease, suggesting a possible link between metabolic disturbances and skin manifestations.
Diagnosis[edit | edit source]
Diagnosis of Kyrle's 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 a central keratotic plug penetrating the epidermis.
Differential Diagnosis[edit | edit source]
The differential diagnosis for Kyrle's disease includes other conditions that present with hyperkeratotic papules, such as:
Treatment[edit | edit source]
Treatment of Kyrle's disease focuses on managing the underlying systemic condition and alleviating symptoms. Topical treatments such as keratolytics (e.g., salicylic acid, urea) and retinoids may be used to reduce hyperkeratosis. In some cases, systemic retinoids or phototherapy may be considered.
Prognosis[edit | edit source]
The prognosis of 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. Management of the associated systemic disease is crucial for improving overall outcomes.
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=263)
NIH genetic and rare disease info[edit source]
Hyperkeratosis follicularis et parafollicularis in cutem penetrans is a rare disease.
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Contributors: Prab R. Tumpati, MD
