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Epidermodysplasia Verruciformis in a Patient with a Renal Transplant: A Rare

Case Report

Article in European Medical Journal · January 2022

DOI: 10.33590/emj/21-00255

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 Epidermodysplasia Verruciformis in a Patient
with a Renal Transplant: A Rare Case Report

Authors: Mahesh Mathur,¹ *Gautam Das,¹ Swati Shah,¹ Sunil Jaiswal,¹ Srijana
Maharjan,¹ Ayasha Shrestha²
1. Department of Dermatology, Venerology and Leprosy, College of Medical Sciences
Hospital, Bharatpur, Nepal
2. Department of Community Medicine, College of Medical Sciences, Bharatpur, Nepal
*Correspondence to gautamdas2344@gmail.com

Received: 29.11.21

Accepted: 24.01.22

Keywords: Acquired, epidermodysplasia verruciformis (EV), renal transplant.

Citation: EMJ. 2022;7[1]:105-108. DOI/10.33590/emj/21-00255. https://doi.org/10.33590/

emj/21-00255.

Abstract
Acquired epidermodysplasia verruciformis is a rare condition that can occur in patients who are
immunocompromised, particularly recipients of a renal transplant. In a patient who has had a renal
transplant, acquired epidermodysplasia verruciformis has a greater propensity for developing
non-melanoma skin cancer. It is critical to emphasise an early and accurate diagnosis, and regularly
monitor this high-risk population.

INTRODUCTION The diagnosis of EV is usually clinical, and it

can be confirmed by specific histopathological
findings and EV-HPV identification using PCR.6
Epidermodysplasia verruciformis (EV) is a rare,
There is currently no effective treatment for
autosomal recessive genodermatosis affecting
EV. However, early diagnosis, sun protection,
the skin. It is characterised by an unusual
life-long monitoring for malignant transformation,
susceptibility to the human papillomavirus
and therapeutic modalities such as acitretin,
(HPV) infection, especially with HPV5 and HPV8,
imiquimod, topical retinoids, cryotherapy, and
without gender and race preponderance.1,2 It can
others are available for the treatment of EV.7
also be acquired and is more common in patients
who are immunocompromised, such as those Only a few cases of acquired EV, especially
who have had a kidney transplant.3 among recipients of a renal transplant, have
been reported in the literature worldwide. The
The clinical feature is characterised by hypo- or
authors report a case of acquired EV in a patient
hyperpigmented macules, skin-coloured flat-
who had a renal transplant, along with a review
topped papules, and plaques with mild scaling
of the literature.
that develop in children over the face, neck,
trunk, and extremities.1,4 According to reports, the
risk of malignant transformation of skin lesions, CASE PRESENTATION
particularly those on sun-exposed areas, is
A 24-year-old female patient who had had a renal
increased by 35–50%.1,5
transplant presented to the dermatology clinic
with asymptomatic hypopigmented macules

Creative Commons Attribution-Non Commercial 4.0 March 2022 • EMJ 105

 with branny scaling and multiple flat-topped On examination, there were numerous
shiny papules over the forehead and cheek hypopigmented macules, with mild scaling as well
bilaterally. She was on systemic corticosteroid as numerous skin-coloured flat-topped papules
and mycophenolate mofetil and had and plaques over the forehead and cheeks on
consanguineous parents. both sides. The size ranged from 0.5 to 1.0 cm,
The lesions started on the forehead, gradually the shape was round to oval, and the surface was
increasing in number to involve the cheek. smooth with normal surrounding areas (Figures
There were no similar skin lesions among 1 and 2). Hair, nail, and mucous membrane were
family members. Photosensitivity, joint pain, not involved, and systemic examination was
skin rashes, oral ulcer, fever, night sweats, normal. The patient’s serological examination
and lymphadenopathy were not present. ruled out HIV infection.

Figure 1: Multiple hypopigmented flat-topped papules and plaques present over the forehead (orange arrows).

Figure 2: Multiple hypopigmented macules with scaling present over the lateral side of the face (orange arrows).

106 EMJ • March 2022 EMJ

 A punch skin biopsy was performed on the keratinocytes in the upper layer, with pale
hypopigmented, slightly scaly macules over bluish cytoplasm. The epidermal cells showed
the forehead, which showed basket-weave no dysplastic changes (Figure 3). Based on
hyperkeratosis; acanthosis; mild papillomatosis; the clinical and histopathological features, a
prominent granular layer with vacuolisation of diagnosis of EV was made. The patient was given
keratinocytes in the upper and lower epidermis; topical tretinoin gel and told to wear sunscreen
and a focal area showing enlarged or swollen on a daily basis, with regular follow-up.

Figure 3: A histologic analysis of the forehead with a punch biopsy showed basket-weave hyperkeratosis,
hypergranulosis, acanthosis, mild papillomatosis, and a large focal area affected with keratinocytes, with
characteristic steel blue–grey cytoplasm, large nuclei, and clumping of keratohyaline granules (blue arrows) in the
upper epidermis (haematoxylin-eosin stain; original magnification: ×40).

DISCUSSION tumour suppressor gene protein (p53), leading

to the development of skin malignancies in adult
EV is a rare, autosomal recessive genodermatosis, patients.8,9 Transformation into non-melanoma
which affects cell-mediated immunity and has skin cancer occurs in 35–50% of patients between
no preference for gender, race, or geographic the ages of 40 and 50.4 Malignant transformation
area.1 There is an increased risk of non-melanoma is caused by infection with specific strains of
skin cancer, mainly squamous cell carcinoma HPV, particularly HPV5 and HPV8. More than 30
(SCC).3 The disease can be sporadic, hereditary, EV-associated HPVs have been identified,
or acquired, with the hereditary form being the including HPV5, HPV8, HPV12, HPV14, HPV15,
most common. The acquired form can be seen HPV17, HPV19, HPV25, HPV36, HPV38, HPV47,
in recipients of a renal transplant, Hodgkin’s and HPV50.3,4,10 Skin malignancies normally
disease, systemic lupus erythematosus, and HIV develop after a long period of time, and they
infection.3 seldom metastasise or penetrate deeper tissues.6

On chromosome 17q25, the EVER1 or EVER2 In patients with EV, hypo- or hyperpigmented
genes have been linked to impaired cell-mediated macules resembling pityriasis versicolor, verruca
immunity and an unusually high susceptibility plana-like lesions, and seborrhoeic keratosis-like
to a specific strain of HPV infection.1 A higher plaques are prevalent. These lesions typically
lifetime cumulative sunlight exposure, X-ray start in childhood and affect the face, neck,
irradiation, and immunologic defects in patients extremities, and trunk, with mucous membrane
with EV are likely to induce mutations of the involvement being rare.1,3,9

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 The clinical and histopathological examinations however, are ineffective against EV. In order to
are used to diagnose EV. All clinical lesions of prevent skin cancer, patients must be educated
EV share common histopathological features, about the disease’s relapsing and persistent
which include basket-weave hyperkeratosis of course, photoprotection, early detection, and
the stratum corneum, parakeratosis, acanthosis, excision of premalignant and malignant lesions
and characteristic cytopathic changes of infected with regular follow-up.3,8
keratinocytes in the malpighian layer. Infected
keratinocytes show characteristic cytopathic CONCLUSION
changes characterised by large cells with
pale blue–grey cytoplasm and variable sized Acquired EV is a rare entity and can be acquired
kerato-hyaline granules. Regardless of the in patients who are immunodeficient, especially
infecting HPV strain, these findings are consistent in those who have had a transplant. Recipients
across all EV-HPV infections.6 of a renal transplant with acquired EV are at
high risk of developing non-melanoma skin
There is currently no effective treatment for cancers.11 As a result, it is critical for the treating
EV. There are, however, certain medical and physician to recognise and accurately diagnose
surgical treatments available. Topical and oral acquired EV as early as possible, and to reduce
retinoids, imiquimod, cimetidine, interferon-α, the risk of early malignant transformation of skin
electrocautery, and cryotherapy are some of lesions through strict sun protection and regular
the options. All of these therapeutic options, follow-up.

References
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