Lutz–Lewandowsky epidermodysplasia verruciformis

Lutz–Lewandowsky epidermodysplasia verruciformis
Synonyms	Tree man syndrome
Pronounce	N/A
Specialty	N/A
Symptoms	Wart-like lesions, skin lesions
Complications	Skin cancer
Onset	Childhood
Duration	Lifelong
Types	N/A
Causes	Genetic mutation
Risks
Diagnosis	Clinical evaluation, genetic testing
Differential diagnosis	N/A
Prevention	N/A
Treatment	Surgical removal, topical treatments
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

Lutz–Lewandowsky epidermodysplasia verruciformis (EV), also known as tree man syndrome, is a rare genetic disorder characterized by an abnormal susceptibility to human papillomavirus (HPV) infections. This condition leads to the development of wart-like lesions on the skin, which can progress to skin cancer in some cases.

History

The condition was first described by the physicians Felix Lewandowsky and Wilhelm Lutz in 1922. They reported a case of a patient with widespread wart-like lesions that were resistant to conventional treatments.

Genetics

Epidermodysplasia verruciformis 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 the skin, and their mutation leads to an impaired immune response to certain types of HPV.

Pathophysiology

The condition is characterized by an increased susceptibility to specific types of HPV, particularly types 5 and 8. These viruses cause the development of flat, wart-like lesions that can cover large areas of the body. Over time, these lesions may undergo malignant transformation, leading to squamous cell carcinoma.

Clinical Presentation

Patients with epidermodysplasia verruciformis typically present in childhood with flat, wart-like lesions on the skin. These lesions are often found on the face, hands, and feet, but can occur anywhere on the body. The lesions are usually asymptomatic but can become disfiguring and may lead to social stigma.

Diagnosis

Diagnosis is primarily clinical, based on the appearance of the lesions and the patient's history. Genetic testing can confirm mutations in the EVER1 or EVER2 genes. Skin biopsy may be performed to rule out malignancy.

Treatment

There is no cure for epidermodysplasia verruciformis, but treatment focuses on managing symptoms and preventing complications. Options include:

• Surgical removal of lesions
• Topical treatments, such as retinoids or imiquimod
• Regular monitoring for signs of skin cancer

Prognosis

The prognosis for patients with epidermodysplasia verruciformis varies. While the condition is lifelong, the risk of developing skin cancer can be managed with regular monitoring and treatment.

Also see

• Human papillomavirus
• Squamous cell carcinoma
• Genetic disorders
• Dermatology

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