Epidermodysplasia verruciformis (also called Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky epidermodysplasia verruciformis) is a rare autosomal recessive genetic hereditary skin disorder associated with a high risk of carcinoma of the skin. It is characterized by abnormal susceptibility to human papillomaviruses (HPVs) of the skin. The resulting uncontrolled HPV infections result in the growth of scaly macules and papules, particularly on the hands and feet. It is typically associated with HPV types 5 and 8, which are found in about 80% of the normal population as asymptomatic infections, although other types may also contribute. but can occasionally present in middle-age.
Patients present with flat, slightly scaly, red-brown macules on the face, neck and body, reoccuring especially around the penial area, or verruca-like papillomatous lesions, seborrheic keratosis-like lesions, and pinkish-red plane papules on the hands, upper and lower extremities, and face. The benign form of EV presents with only flat, wart-like lesions over the body, whereas the malignant form shows a higher rate of polymorphic skin lesions and development of multiple cutaneous tumors.
Generally cutaneous lesions are disseminated over the body, but there are some cases with only a few lesions which are limited to one extremity.
Interferons can also be used effectively together with retinoids.
Cimetidine was reported to be effective because of its depressing mitogen-induced lymphocyte proliferation and Regulatory T cell activity features. A report by Oliveira et al. showed that cimetidine was ineffective. Hayashi et al. applied topical calcipotriol to a patient with a successful result.
As mentioned, various treatment methods are offered against EV; however, most importantly, education of the patient, early diagnosis and excision of the tumoral lesions take preference to prevent the development of cutaneous tumors.
In November 2007, a new video of a 34-year-old Indonesian man named Dede Koswara with a similar disease appeared on the internet. His story has appeared in the Discovery Channel and TLC series "My Shocking Story" ("Extraordinary People" on UK's Five) in the episode "Half Man Half Tree". And then on August 12, 2008, Dede Koswara's story was the subject of an ABC's Medical Mystery episode entitled "Tree Man".
On 26 August 2008, Dede returned home following surgery to remove of warts from his body. The surgery consisted of three steps:
* Removal of the thick carpet of warts and massive horns on his hand.
In all, 95% of the warts were removed. The Discovery Channel funded a blood analysis and found he lacked an immune system antigen to fight yeast infection. He was offered to have more tests run to determine whether it is treatable, and the doctor was fairly optimistic, but he refused the treatment.
In 2009 the Discovery Channel episode "Treeman Meets Treeman" reported on Lin Tianzhuan, who also has the disease and was given a similar treatment for it. Thus far his treatment seems to have worked better. In the Netherlands a man of Indonesian ethnicity also had the disease and was treated with radiation therapy, which seemed to be successful but gave him crippling cancer.
Additionally, a similar case was profiled on season 5, episode 20 of Nip/Tuck.
Category:Virus-related cutaneous conditions Category:Rare diseases Category:Viral diseases Category:Epidermal nevi, neoplasms, cysts
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