Indian J Sex Transm Dis Indian J Sex Transm Dis
Official Publication of the Indian Association for the Study of Sexually Transmitted Diseases
Indian J Sex Transm Dis
The Journal | Search | Ahead Of Print | Current Issue | Archives | Instructions | Subscribe | Login    Users online: 266   Home Email this page Print this page Bookmark this page Decrease font size Default font size Increase font size


 
  Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 41  |  Issue : 1  |  Page : 105-107
 

Epidermodysplasia verruciformis with plane warts over lower abdomen and genitals


Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad, Gujarat, India

Date of Submission22-Dec-2016
Date of Acceptance25-Feb-2020
Date of Web Publication18-Jun-2020

Correspondence Address:
Dr. Pragya Ashok Nair
Department of Dermatology and Venereology, Pramukshwami Medical College, Karamsad - 388 325, Gujarat
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijstd.IJSTD_110_16

Rights and Permissions

 

   Abstract 

Epidermodysplasia verruciformis (EDV) may clinically vary from pityriasis versicolor-like macules to wart-like flat papules, psoriasiform red papules, or pigmented keratotic lesions resembling seborrheic keratosis. Sun-exposed areas are commonly affected with genital areas rarely involved. It is associated with more than 30 human papillomaviruses (HPVs). In 90% cases of squamous cell carcinomas, HPV5 and HPV8 is isolated. A case of EDV with plane warts involving the genital area in a 35-year-old male is reported here.


Keywords: Epidermodysplasia verruciformis, genitals, human papillomavirus, warts


How to cite this article:
Singhal RR, Sheth NK, Nair PA. Epidermodysplasia verruciformis with plane warts over lower abdomen and genitals. Indian J Sex Transm Dis 2020;41:105-7

How to cite this URL:
Singhal RR, Sheth NK, Nair PA. Epidermodysplasia verruciformis with plane warts over lower abdomen and genitals. Indian J Sex Transm Dis [serial online] 2020 [cited 2020 Sep 18];41:105-7. Available from: http://www.ijstd.org/text.asp?2020/41/1/105/286998



   Introduction Top


Epidermodysplasia verruciformis (EDV) is a rare inherited disorder that predisposes the patients to widespread human papillomavirus (HPV) infection. Cutaneous lesions may vary from pityriasis versicolor-like macules to wart-like flat papules, psoriasiform red papules, and pigmented keratotic lesions resembling seborrheic keratosis. EDV is associated with more than 30 HPVs. HPV5 and HPV 8 are associated with Squamous cell carcinoma in 90% cases. The lesions mainly occur on the sun-exposed areas, and genital areas are rarely involved. Genital warts in patients with EDV are rare, with only four cases reported till date.[1],[2],[3],[4] We describe here an unusual case of a EDV presenting with pigmented seborrheic keratosis-like lesions and plane warts, both together over genital areas.


   Case Report Top


A 35-year-old married male presented to the dermatology department with a 4-year history of multiple lesions over the abdomen, left side of the penis, and scrotum, which were asymptomatic. The lesions were nonprogressive in nature. There was a history of the same type of lesions present in the wife. There were no complaints of ulcer, any discharge, urinary tract infection, abdominal pain, or fever. There was a history of infertility present in the couple. On examination multiple hyperpigmented dome shaped papules over lower abdomen. [Figure 1]a and some warty papules present over penis and scrotum. [Figure 1]b There were no hypopigmented macules over any part of body. Systemic examination showed no abnormalities. Routine laboratory tests including hematological and biochemical analyses of blood and urine were within normal ranges, and serology for HIV and syphilis was negative. Biopsy from dome-shaped plane papule over the lower abdomen was taken keeping bowenoid papulosis, seborrheic keratosis, and warts as differential. Histopathology showed hyperkeratosis, acanthosis, and vacuolated cells in the upper stratum malpighi and granular layer. Keratinocytes have basophilic cytoplasm containing keratohyalin granules, suggestive of EDV [Figure 2].
Figure 1: (a) Multiple hyperpigmented dome-shaped papules over the lower abdomen, (b) multiple flat wart-like lesions over the penis and scrotum

Click here to view
Figure 2: Hyperkeratosis, acanthosis, and vacuolated cells in the upper stratum malpighiand granular layer keratinocytes have basophilic cytoplasm containing keratohyalin granule (H and E, ×4)

Click here to view


Biopsy from warty lesion over the scrotum revealed hyperkeratosis and acanthosis. The stratum malphigii showed papillomatosis. The rete ridges were elongated and bent inward at both margins, and large vacuolated cells were seen in the upper stratum malpighisuggestive of warts. Dermis showed mild perivascular lymphoplasmacytic infiltration [Figure 3]. The patient was advised for cryotherapy which was given weekly for 12 weeks and all the lesions subsided. The patient was not advised oral retinoids as there was a history of infertility, and the couple was taking treatment for the same.
Figure 3: Hyperkeratosis, acanthosis, and papillomatosis. The rete ridges were elongated and bent inward at both margins with large vacuolated cells in the upper stratum malpighi (H and E, ×4)

Click here to view



   Discussion Top


EDV was first described by Lewandowsky and Lutz in 1922 as an epidermal nevus.[5] It is a rare, generalized, persistent condition with a defective cell-mediated immune response to HPV infection and may be inherited or acquired. The inherited form usually presents in infancy or early childhood and is usually autosomal recessive, though autosomal dominant and X-linked dominant forms have also been reported. Two loci on chromosomes 17q25 and 2p21–p24 are associated with the disease in some families. Mutations in two genes EVER 1 and EVER 2 are linked with the disease in many but not all cases.[6] The acquired type may be secondary in immunocompromised individuals, including those with T-cell lymphoma and HIV. In our patients, the lesions developed at the age of 35 years, so it is likely to be acquired type.

There are at least 30 HPV types with a characteristic of EDV being HPVs 5, 8, 9, 12, 14, 15, 17, and 19–25. The types found more rarely include HPVs 28, 36, 37, 38, 47, 49, 50, 75, 76, 77, 93, and 96. The lesions mainly occur on the sun-exposed area but may be generalized all over the body. It is a rare, autosomal recessive genodermatosis that predisposes patients to widespread HPV infections that do not regress due to unique susceptibility to specific HPV types. Lesions typically have either the appearance of flat warts or scaly, red-brown macules that resemble lesions of pityriasis versicolor.[7]

The development of genital warts in patients with EDV is rare, with only four cases reported till date [Table 1] Rogers et al. reported the development of EDV lesions in two HIV-positive immunocompromised patients.[3] Our patient presented with dome-shaped papules mimicking seborrheic keratosis and warts. The localizations of the lesions are predominantly sun-exposed areas such as the face, neck, and dorsa of the hands. However, warty lesions involving the scrotum and penis may also rarely occur[2] as reported in our case. Oral mucosa is spared. Malignant change occurs in 20%–30% of cases mainly on sun-exposed areas, and metastasis is uncommon. Carcinogenic risk is determined by HPV infection, relative immunosuppression, and sunlight exposure.
Table 1: Reported cases of epidermodysplasia verruciformis and genital wart

Click here to view


The histological picture is similar in the different clinical types of lesion of EDV. Skin biopsy of EDV shows swollen keratinocytes with vacuolation that contains basophilic granules, whereas hyperkeratosis, acanthosis, with a characteristic feature of koilocytosis (vacuolation of keratinocytes) of upper keratinocytes, and papillomatosis along with incurving of rete ridges are the main features of warts. Other differentials include seborrheic keratosis which shows basaloid cell proliferation with horn cysts as a characteristic feature in histopathology, whereas bowenoid papulosis shows windblown appearance of large atypical keratinocytes.

There is no effective treatment for EDV. Etretinate 1 mg/kg/day is helpful in some cases, but the lesions recur. Oral isotretinoin is also effective. In our patients, we had used liquid nitrogen to destroy the lesions. Topical imiquimod, calcipotriol, and squaric acid dibutyl ester are the other treatment options that can be used. Sun protection measures are essential to prevent neoplasia if lesions are over sun-exposed areas.


   Conclusion Top


EDV can mimic seborrheic keratosis and warts and can involve even genitalia. Therefore, biopsy should be taken to confirm the diagnosis at the earliest in case of any dilemma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Kivanc-Altunay I, Erdogan HK, Kayaoglu S. Perianal warts and the development of squamous cell carcinoma in epidermodysplasia verruciformis. Indian J Dermatol Venereol Leprol 2011;77:112.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Sanclemente G, García JJ, Gómez LF, Tyring SK, Wolff JC, Correa LA, et al. An unusual presentation of human papillomavirus (HPV) infection in a black epidermodysplasia verruciformis (EDV) patient. Int J Dermatol 2007;46:199-201.  Back to cited text no. 2
    
3.
Rogers HD, Macgregor JL, Nord KM, Tyring S, Rady P, Engler DE, et al. Acquired epidermodysplasia verruciformis. J Am Acad Dermatol 2009;60:315-20.  Back to cited text no. 3
    
4.
Zhu D, Fang H, Qiao J, Liu X, Xu B, Si Z. Giant warts in a Chinese with epidermodysplasia verruciformis. Indian J Dermatol Venereol Leprol 2011;77:730.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Bogdan I, Schärer L, Rüdlinger R, Hafner J. Epidermodysplasia verruciformis in two brothers developing aggressive squamous cell carcinoma. Dermatol Surg 2007;33:1525-8.  Back to cited text no. 5
    
6.
Zavattaro E, Azzimonti B, Mondini M, De Andrea M, Borgogna C, Dell'Oste V, et al. Identification of defective Fas function and variation of the perforin gene in an epidermodysplasia verruciformis patient lacking EVER 1 and EVER 2 mutations. J Invest Dermatol 2008;128:732-5.  Back to cited text no. 6
    
7.
Gül U, Kiliç A, Gönül M, Cakmak SK, Bayis SS. Clinical aspects of epidermodysplasia verruciformis and review of the literature. Int J Dermatol 2007;46:1069-72.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

Top
Print this article  Email this article
 

    

 
  Search
 
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,468 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed196    
    Printed16    
    Emailed0    
    PDF Downloaded21    
    Comments [Add]    

Recommend this journal