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Epidermodysplasia verruciformis atlasgeneticsoncology.org | Epidermodysplasia verruciformis. DermNet NZ dermnetnz.org |
Epidermodysplasia verruciformis (also called Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky epidermodysplasia verruciformis) is an extremely rare autosomal recessive genetic[1] 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.[2] 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,[3] which are found in about 80% of the normal population as asymptomatic infections,[4] although other types may also contribute.[3] The condition usually has an onset of between the ages of 1–20,[5] but can occasionally present in middle-age.[5] It is named after the physicians who first documented it, Felix Lewandowsky and Wilhelm Lutz.[6]
[edit] Genetic causeThe cause of the condition is an inactivating mutation in either the EVER1 or EVER2 genes, which are located adjacent to one another on chromosome 17.[1] The precise function of these genes is not yet fully understood, but they play a role in regulating the distribution of zinc in the cell nucleus. It has been shown that zinc is a necessary cofactor for many viral proteins, and that the activity of EVER1/EVER2 complex appears to restrict the access of viral proteins to cellular zinc stores, limiting their growth.[7] [edit] DiagnosisClinical diagnostic features are lifelong eruption of pityriasis versicolor-like macules, flat wart-like papules and development of cutaneous carcinomas. Patients present with flat, slightly scaly, red-brown macules on the face, neck and body, 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.[8][9] [edit] TreatmentA totally effective treatment method against EV has not yet been found. Several treatments have been suggested, and acitretin 0.5–1 mg/day for 6 months’ duration is the most effective treatment owing to antiproliferative and differentiation-inducing effects. 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. [edit] Notable casesIn March 2007, a Romanian man named Ion Toader was diagnosed with this condition.[10] The man, a patient of dermatologist Carmen Madeleine Curea, his pictures appeared on numerous blogs and Romanian press sources. Curea works out of Spitalului Clinic Colentina in Bucharest, Romania. Stephen Stone, past president of the American Academy of Dermatology, confirms this is Lewandowsky-Lutz.[citation needed] In November 2007, a new video of a 34-year-old Indonesian man named Dede Koswara with a similar disease appeared on the internet.[11] 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".[12] And then on August 12, 2008, Dede Koswara's story was the subject of an ABC's Medical Mystery episode entitled "Tree Man".[citation needed] On August 26, 2008, Dede returned home following surgery to remove 6 kg (13 lb) of warts from his body.[13] The surgery consisted of four steps:
In all, 95% of the warts were removed.[13] The surgery was documented by the Discovery Channel and TLC in the episode "Treeman: Search for the Cure."[citation needed] However, his warts have returned and it appears he will need two surgeries a year for the rest of his life to keep them at bay.[14] In 2009 the Discovery Channel episode "Treeman Meets Treeman" reported on another Indonesian man, from the same region as Dede, who also has the disease and was given a similar treatment for it. Thus far his treatment seems to have worked better. In Holland a man of Indonesian ethnicity also had the disease and was treated with radiation treatment, which seemed to be successful but gave him crippling cancer.[citation needed] [edit] See also[edit] References
[edit] Further reading
[edit] External links
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