Epidermodysplasia verruciformis

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Classification according to ICD-10
B07 Viral warts
ICD-10 online (WHO version 2019)

Epidermodysplasia verruciformis ( EV , even Lewandowsky-Lutz dysplasia or Lutz-Lewandowsky epidermodysplasia verruciformis ) is an extremely rare autosomal - recessive genodermatosis (hereditary skin disease), which with a high skin cancer is connected -Risk. The skin is unusually susceptible to human papillomavirus (HPV). The resulting uncontrolled HPV infections result in the growth of scaly patches of skin and papules, especially on the hands and feet. It is primarily HPV types 5 and 8 that are asymptomatic (“without symptoms”) in around 80% of the population, but other HPV types can also be involved.

Abul Bajandar from Bangladesh

The disease usually appears between the ages of 1 and 20 years, but can occasionally only manifest in middle age. It got its name from the two doctors who first described it, Felix Lewandowsky and Wilhelm Lutz .

Stefania Jabłońska and Gérard Orth received the 1985 Robert Koch Prize for investigations into papilloma viruses using the example of the EV .

Genetic cause

The cause of the disease is a mutation which inactivates either the gene product of EVER1 ( TMC6 , i.e. transmembrane channel-like protein 6 ) or that of EVER2 ( TMC8 , i.e. transmembrane channel-like protein 8 ). The two genes in question are next to each other on chromosome 17 . The exact function of these genes is not yet fully understood, but they play a role in the regulation of zinc in the cell nucleus . It was shown that zinc is a necessary cofactor for many viral proteins and that the biological activity of the transcription products of EVER1 / EVER2 can reduce the availability of zinc for foreign proteins (i.e. viral proteins) and thus prevent viral growth and spread of the viruses.

diagnosis

In clinical diagnosis, the disease manifests itself as pityriasis versicolor -like spots, flat warty papules and carcinomas of the skin. The patients show flat, slightly scaly, red-brown spots on the face, neck and body, or wart-like lesions as in papillomas or as in seborrheic keratosis and pink-red papules on the hands, upper and lower extremities and face. Generally, the skin lesions are common all over the body, but there are some cases with only a few changes that are confined to one limb.

The benign (i.e. benign) form of epidermodysplasia verruciformis is only manifested by flat, wart-like lesions on the body, while the malignant (i.e. malignant) form is characterized by a higher rate of polymorphic skin changes and the development of various types of skin cancers .

therapy

There is currently no long-term successful therapy for epidermodysplasia verruciformis . It is important to have an early diagnosis and, in addition to medical treatment, that the patient learns to deal with his illness. In most cases, tumoral lesions are surgically removed to prevent skin cancer from developing.

Various treatments have been suggested; the administration of the keratolytic retinoid acitretin (0.5 to 1 mg / day for six months) is currently the most effective treatment. Also interferons - together with retinoids - are intended to provide a successful treatment. The effect of cimetidine is controversial: It has been reported that because of its effect, mitogen -induced lymphocyte - growth to suppress, was effective. A report by Oliveira et al. Suggests that cimetidine is ineffective. Hayashi et al. Achieved positive results in one patient from topical application of calcipotriol .

literature

Individual evidence

  1. ↑ a b N. Ramoz, LA Rueda, B. Bouadjar et al .: Mutations in two adjacent novel genes are associated with epidermodysplasia verruciformis . In: Nature Genetics . tape 32 , no. 4 , December 2002, p. 579-581 , doi : 10.1038 / ng1044 , PMID 12426567 .
  2. ↑ M. Lazarczyk, C. Pons, JA Mendoza et al .: Regulation of cellular zinc balance as a potential mechanism of EVER-mediated protection against pathogenesis by cutaneous oncogenic human papillomaviruses . In: Journal of Experimental Medicine . tape 205 , no. 1 , January 2008, p. 35–42 , doi : 10.1084 / jem.20071311 , PMID 18158319 , PMC 2234378 (free full text).
  3. ↑ a b G. Orth: Epidermodysplasia verruciformis: a model for understanding the oncogenicity of human papillomaviruses . In: Ciba Foundation Symposium . tape 120 , 1986, pp. 157-174 , PMID 3013521 .
  4. ↑ A. Antonsson, O. Forslund, H. Ekberg et al .: The ubiquity and impressive genomic diversity of human skin papillomaviruses suggest a commensalic nature of these viruses . In: Journal of Virology . tape 74 , no. December 24 , 2000, pp. 11636–11641 , doi : 10.1128 / JVI.74.24.11636-11641.2000 , PMID 11090162 , PMC 112445 (free full text).
  5. ↑ a b U. Gül, A. Kiliç, M. Gönül et al .: Clinical aspects of epidermodysplasia verruciformis and review of the literature . In: International Journal of Dermatology . tape 46 , no. 10 , 2007, p. 1069-1072 , doi : 10.1111 / j.1365-4632.2006.03014.x , PMID 17910717 .
  6. ^ Lewandowsky-Lutz dysplasia: Who Named It? On: whonamedit.com
  7. ↑ M. Lazarczyk, M. Favre: Role of Zn2 + ions in host-virus interactions . In: Journal of Virology . tape 82 , no. December 23 , 2008, pp. 11486–11494 , doi : 10.1128 / JVI.01314-08 , PMID 18787005 , PMC 2583646 (free full text).
  8. ↑ DR Lowy, EJ Androphy: Fitzpatrick's Dermatology in General Medicine . Ed .: IM Freedberg, AZ Eisen, K. Wolff et al. 6th edition. McGraw-Hill , New York City 2003, ISBN 978-0-07-138076-8 , Warts, pp. 2119-2131 .
  9. ↑ Walmar Roncalli Pereira de Oliveira, Solange Carrasco, Cyro Festa Neto, Peter Rady, Stephen K. Tyring: Nonspecific cell-mediated immunity in patients with epidermodysplasia verruciformis . In: The Journal of Dermatology . tape 30 , no. 3 , March 2003, p. 203-209 , PMID 12692356 ( dermatol.or.jp ). Nonspecific cell-mediated immunity in patients with epidermodysplasia verruciformis ( Memento of the original dated February 4, 2010 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice.  @1@ 2Template: Webachiv / IABot / www.dermatol.or.jp

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