Epidermodysplasia verruciformis

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Epidermodysplasia verruciformis Classification and external resources
OMIM	226400 305350
DiseasesDB	31394
eMedicine	derm/123

Epidermodysplasia verruciformis (also called Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky epidermodysplasia verruciformis) is an extremely rare skin disease characterized by the growth of scaly macules and papules, particularly on the hands and feet. Named after doctors Felix Lewandowsky and Wilhelm Lutz,^[1] the condition usually begins in childhood and has no known cure, although high doses of vitamin A may ameliorate the condition.

One case study stated that the onset age is between 1-20.^[2] However, it is also possible that the disease begins in middle-age.^[2]

It is believed to be caused by the human papillomavirus and is most strongly associated with strains 5 and 8, although others may also contribute. Genetic predisposition (Autosomal Recessive) is also thought to partly explain why the disease is so rare, as EV papillomaviruses are often found (15-20%) in normal skin with no increased incidence of cancer.

1 Diagnosis
2 Treatment
3 Notable cases
4 References
5 Further reading
6 External links

[edit] Diagnosis

Clinical diagnostic features are lifelong eruption of pitriasis 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; however, there are some cases with only a few lesions which are limited to one extremity.^[3]^[4]

[edit] Treatment

A totally effective treatment method against EV has not yet been found. However, 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. However, a report by Oliveria 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 cases

In March 2007, a romanian man named Ion Toader was diagnosed with this condition.^[5] The man, a patient of dermatologist Dr. Carmen Madeleine Curea, quickly became an internet meme, his pictures appearing on numerous blogs and Romanian press sources. Dr. 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, Dede Koswara with a similar disease appeared on the internet.^[6] 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".^[7]

[edit] References

^ Lewandowsky-Lutz dysplasia: Who Named It?
^ ^a ^b Clinical aspects of epidermodysplasia verruciformis and review of the literature by Ülker Gül, MD, Arzu Kılıç, MD, Müzeyyen Gönül, MD, Seray Külcü Çakmak, MD, and Seçil Soylu Bayis. International Journal of Dermatology Vol. 46, Issue 10, Page 1069-1072. October 2007.
^ Lowy DR, Androphy EJ. Warts. In: Freedberg IM, Eisen AZ, Wolff K, et al. 6th edn. New York: Mc Graw-Hill, 2003: 2119–2131.
^ Pereira de Oliveira WR, Carrasco S, Neto CF, et al. Nonspecific cell-mediated immunity in patients with epidermodysplasia verruciformis. J Dermatol 2003; 30: 203–209.
^ WFMU's Beware of the Blog: Missionary encounters extremely bizarre skin condition in Eastern Europe
^ The man who looks like a tree | Metro.co.uk
^ My Shocking Story: Half Man Half Tree. Discovery Channel.

[edit] Further reading

H. Sadakane, Y. Yabe. The Virus of Epidermodysplasia Verruciformis: Electron Microscopic and Fluorescent Antibody Studies. Journal of Investigative Dermatology (1975) 65, 324–330 [1]
F. Lewandowsky, W. Lutz. Ein Fall einer bisher noch nicht beschriebenen Hauterkrankung (Epidermodysplasia verruciformis). Archiv für Dermatologie und Syphilis (1922) 141, 193-202.