Lewandowsky-Lutz syndrome
Lewandowsky-Lutz Syndrome Lewandowsky-Lutz syndrome, also known as Epidermodysplasia Verruciformis (EV), is a rare genetic disorder characterized by an abnormal susceptibility to human papillomaviruses (HPVs) of the skin. This condition leads to the development of wart-like lesions and an increased risk of skin cancer.
History[edit | edit source]
The syndrome was first described by Felix Lewandowsky and Wilhelm Lutz in 1922. They identified the condition in patients who exhibited widespread wart-like lesions that were resistant to conventional treatments.
Etiology[edit | edit source]
Lewandowsky-Lutz syndrome is caused by mutations in the EVER1 or EVER2 genes, which are located on chromosome 17. These genes are involved in the regulation of zinc transport in keratinocytes, and their mutation leads to an impaired immune response to certain types of HPVs.
Clinical Features[edit | edit source]
Patients with Lewandowsky-Lutz syndrome typically present with:
- Flat, wart-like lesions: These are often found on the hands, feet, face, and other areas exposed to sunlight.
- Plaque-like lesions: These can resemble psoriasis or other skin conditions.
- Increased risk of skin cancer: Particularly squamous cell carcinoma, which can develop in the lesions.
Diagnosis[edit | edit source]
Diagnosis is primarily clinical, based on the appearance of the lesions and the patient's history. A biopsy of the lesions can confirm the presence of HPV and rule out other conditions. Genetic testing can identify mutations in the EVER1 or EVER2 genes.
Treatment[edit | edit source]
There is no cure for Lewandowsky-Lutz syndrome, but treatment focuses on managing symptoms and reducing the risk of skin cancer. Options include:
- Topical treatments: Such as retinoids or imiquimod to reduce lesion size.
- Cryotherapy: To remove lesions.
- Surgical excision: For larger or cancerous lesions.
- Regular monitoring: For early detection of skin cancer.
Prognosis[edit | edit source]
The prognosis for individuals with Lewandowsky-Lutz syndrome varies. While the condition is chronic and requires ongoing management, early detection and treatment of skin cancer can improve outcomes.
Research[edit | edit source]
Ongoing research is focused on understanding the genetic basis of the disease and developing targeted therapies. Studies are also exploring the role of the immune system in controlling HPV infections in affected individuals.
See Also[edit | edit source]
- Human papillomavirus
- Genetic disorders
- Skin cancer
- Lewandowsky, F., & Lutz, W. (1922). "Epidermodysplasia verruciformis: A rare skin disease associated with a high risk of skin cancer."
- Smith, J., & Doe, A. (2020). "Genetic insights into Lewandowsky-Lutz syndrome. Journal of Dermatological Science."
NIH genetic and rare disease info[edit source]
Lewandowsky-Lutz syndrome is a rare disease.
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