Epidermodysplasia verruciformis – how much do we know about it?

Papillomavirus, a tree-like man, hares with horns… What connects these concepts? This is probably Lewandowsky – Lutz dysplasia. Probably because little is known about the disease for the time being. We know how does it look, we suppose what it’s etiology is and the treatment is limited to cosmetic procedures. This paper aims to garner the known facts about this surprising disease entity.

Lewandowsky – Lutz dysplasia is a type of dermatological disease manifested by the appearance of the different types of skin lesions. It is believed that the HPV – human papillomavirus (mainly serotypes 5, 8 and 14 ) is responsible for this condition. In people with a genetic predisposition the mutation of chromosome 17 occur within two genes – EVER2 or EVER1. Indeed it is certain that this predisposition is genetically determined and inherited in an autosomal recessive way. The genetic defect in people consist in lowering the immunological potential of the skin, whereby it comes to the penetration of the virus into the deep layers and the emergence of clinical symptoms. Virus (in favourable genetical conditionsis) is tolerated by the immune system.

A full description of the disease was first presented in 1922 by dermatologists Felix Lewandowsky (1879-1921) and Wilhelm Lutz (1888-1952) in the pages of the publication of Berlin’s ‘Archiv für Dermatologie und Syphilis’. Both men have contributed extensively to the development of the modern dermatology and venereology.

The condition is rare in a way that there is no more than five affected for 10 thousand people. It applies to all age groups including children but the largest proportion is in patients aged 25-58 years (average 39 years). The risk factors which predispose and accelerate the development of the disease are infections with HIV and AIDS, organ transplants and other conditions associated with long-term immunosuppression. Taking into account the etiological factor and ways of infection, it is assumed that over the half of human population underwent the infection with the human papillomavirus asymptomatically or mildly symptomatically. The virus is carried by the contact with the skin, sexually or from mother to child during childbirth. In many people, HPV is in the stage of latency which means that it does not exposure and due to the action of the immune system infection recedes idiopathically. In the cases of people showing signs of immunosuppresion chronic infection with HPV may occur and consequently lead to the formation of skin lesions and cancer.

Epidermodysplasia verruciformis symptoms are very diverse. Skin lesions involve usually exposed parts of the body such as the face (primarily the forehead), the dorsal part of the hands, forearms or lower leg area. Symptoms do not apply to mucous membranes and the products of the epidermis (hair, nails). The appearance of skin lesions can be different- from the simple and benign as erythema and hyperpigmentation to flat warts resembling lumps of different colors. Therefore, the EV in the early stages is confused with diseases such as flat warts (verrucae planae) or dandruff versicolor (pityriasis versicolor). The disease is not curable, and the changes that occurred in childhood worsen throughout life. EV pharmacotherapy involves administration of drugs from the group of retinoids (vitamin A and its derivatives – inhibit the transcription of genes of the virus), which in some cases regress changes temporarily or only inhibit their development bringing temporal improvement. The surgical therapy or laser treatments are also not effective forms of disease control. Deleted changes frequently recur or turn into skin cancers such as c. spinocellulare or c. basicellulare. Due to the acceleration of the disease process by ultraviolet radiation, there is a need to apply protective creams with high sunscreen during the early years of the disease.

The most famous case of papillary epithelial dysplasia is Dede Koswara, known as a tree- man. Dede Koswara, who is Indonesian in origin, is suffering from many years on the variety of ailments that doctors interpret as an EV. His body is almost entirely covered in thick layer of papules structurally similar to the bark of a tree. His pathologically changed hands look like branches.

Usually the disease does not manifest so quickly and does not give such big changes but in the case Koswara changes are so extensive that virtually preclude him from everyday life. Dede must have surgery every six months for normal existence. Doctors do not give him hope. After the last visit of Koswara in hospital, doctors removed 6 kg of warts from his body. Indonesian, when asked about how did he become sick, claims that he cut himself with a knife when scaling a fish. He believes that at this moment he contracted the disease since the virus might gain entry to his bloodstream.

Other cases of this disease include a man from Romania, and two cases from China, (for example 38-year-old Lin Thianzhuan). Moreover, animals are not immune to the Lewandowsky-Lutz disease. In various countries of the world it is sometimes said that there are strange creatures i.e. Loch Ness monster, Yeti or the horned hare (Lepus cornutus). Despite the fact that the last-mentioned one was associated with the hybrid of a rabbit and an antelope (so called Jackalope), these stories may be true because animals are affected with the disease as are people. A study of real horned hares helped to isolate CRPV virus (Cottontail Rabbit Papilloma Virus), which is the equivalent of human HPV. Perhaps they suffer from a variety of animal EV? Maybe the legendary unicorns were just sick horses?

To sum up, epidermodysplasia verruciformis is a disease which is still identified. Drugs are constantly being tested and the scientist are constantly trying to prove more facts about the disease. But let’s hope that modern medicine will cope with the task and once we can effectively prevent this very strange disease.

Written by: Mateusz Grajek, Anna Rej, Sandra Kryska

Source:
1. Epidermodysplasia verruciformis, www.gen.org.pl [on-line: 21.08.11]
2.Epidermodysplasia verruciformis, www.emedicine.medscape.com [on-line21.08.11]
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7. Lewandowsky-Lutz dysplasia, www.whonamedic.com [on-line: 21.08.11]
8. Felix Lewandowsky, www.whonamedic.com [on-line: 21.08.11]
9. Wilhelm Luts, www.whonamedic.com [on-line: 21.08.11]
10. Rogate zające, człowiek-drzewo i HPV, www.migg.wordpress.com [on-line: 21.08.11]

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