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Case Reports
. 2010 Spring;18(1):21-4.

Epidermodysplasia verruciformis: An early and unusual presentation

Ilteris Murat Emsen  1 , M Esref Kabalar
Affiliations
Case Reports

Epidermodysplasia verruciformis: An early and unusual presentation

Ilteris Murat Emsen et al. Can J Plast Surg. 2010 Spring.

Abstract
in English, French

Epidermodysplasia verruciformis is a rare, heritable disease characterized by an unusual susceptibility to infection with specific types of human papillomavirus and a propensity for developing malignant skin tumours. Partial defects in cell-mediated immunity have been demonstrated in this disease. In the present report, a young patient with epidermodysplasia verruciformis who was treated successfully with surgical treatment is described.

L’épidermodysplasie verruciforme est une maladie congénitale rare caractérisée par une susceptibilité inhabituelle à l’infection par des types précis de papillomavirus humains et une propension à développer des tumeurs cutanées malignes. Des anomalies partielles de l’immunité à médiation cellulaire sont démontrées dans cette maladie. Dans le présent rapport, on décrit le cas d’une jeune patiente atteinte d’épidermodysplasie verruciforme dont le traitement chirurgical a réussi.

Keywords: Cell-mediated immunity; Epidermodysplasia verruciformis; Human papillomavirus.

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Figures

Figure 1)
Figure 1)
Histological sections of the lesion showing a hyperkeratotic and slightly acanthotic epidermis. Vacuola cells are present in the upper stratum malpighii and granula layer (hematoxylin and eosin stain, original magnification ×100 [A]; hematoxylin and eosin stain, original magnification ×200 [B]; hematoxylin and eosin stain, original magnification ×400 [C])
Figure 2)
Figure 2)
Multiple hyperkeratotic, pinkish-red papillomatous lesions and tan-coloured macular lesions on her hands
Figure 3)
Figure 3)
Preoperative frontal view of the scarred mass
Figure 4)
Figure 4)
Preoperative lateral view of the mass
Figure 5)
Figure 5)
Preoperative design of the V-Y and Mustarde flaps
Figure 6)
Figure 6)
Perioperative view of the prepared flaps and after removed mass
Figure 7)
Figure 7)
Cosmetic result 14 months after surgery
Figure 8)
Figure 8)
Closer view after surgery
See this image and copyright information in PMC

References

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    1. Ramoz N, Taieb A, Rueda L, et al. Evidence for a nonallelic heterogeneity of epidermodysplasia verruciformis with two susceptibility loci mapped to chromosome regions 2p21–p24 and 17q25. J Invest Dermatol. 2000;114:1148–53. - PubMed
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