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Case Reports
. 2025 Jan 27;17(1):e78058.
doi: 10.7759/cureus.78058. eCollection 2025 Jan.

Epidermodysplasia Verruciformis: A Case Series

Priyanka Rm  1 , Manuvidhya H  1 , Anuradha Priyadarshini  1 , Leena Dennis Joseph  2 , Adikrishnan Swaminathan  1
Affiliations
Case Reports

Epidermodysplasia Verruciformis: A Case Series

Priyanka Rm et al. Cureus. .

Abstract

Epidermodysplasia verruciformis (EV) is an uncommon disease that is inherited autosomal recessively. It has a heightened vulnerability to human papillomavirus (HPV) infection, which manifests as hyperpigmented or hypopigmented macular lesions, lesions resembling pityriasis versicolor (PV) and planar warts, and an increased risk of progressing to skin cancer, especially in sun-exposed areas. We report three cases of EV with varying presentations. One patient, a long-standing case of EV, developed squamous cell carcinoma (SCC) in the vulva and inguinal region. Another patient had a rare association with palmar pits, while the third patient presented with classical EV. All our patients were prescribed oral retinoids and advised to practice stringent photoprotection after histopathology confirmed evidence of EV.

Keywords: edv; epidermodysplasia verruciformis; ev; hpv; lewandowski-lutz dysplasia; squamous cell carcinoma; vulval carcinoma.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Clinical photo showing multiple well defined PV-like lesions on the trunk
PV, pityriasis versicolor
Figure 2
Figure 2. Clinical photo showing wart-like lesions on the neck
Figure 3
Figure 3. Clinical photo showing a large, single, non-healing, tender ulcer, with rolled-out edges in the right inguinal fossa
Figure 4
Figure 4. Dermascopic photo showing continuous marginal scaling
Figure 5
Figure 5. Dermascopic photo showing brownish-black areas mixed with hypopigmented area
Figure 6
Figure 6. H&E stain of skin biopsy from the right arm under 400x magnification, showing epidermis with few keratinocytes with steel-grey cytoplasm, nuclear inclusions, and prominent basophilic granules
H&E, hematoxylin and eosin
Figure 7
Figure 7. H&E staining of skin biopsy from the ulcer
Figure (A) (40x magnification) and Figure (B) (400x magnification) show skin with focal ulceration and underlying subepithelium showing infiltration nests of moderately pleomorphic cells. The individual cells are large, polygonal, with indistinct cell borders, abundant eosinophilic cytoplasm, and round to oval vesicular nuclei with prominent nucleoli. Numerous atypical mitotic figures (black square) are noted. Individual cell keratinization and keratin pearl formation (black circle) are also seen. H&E, hematoxylin and eosin
Figure 8
Figure 8. Clinical photo showing multiple well defined PV-like lesions on the trunk
PV, pityriasis versicolor
Figure 9
Figure 9. Clinical picture showing palmar pits in an EV patient
EV, epidermodysplasia verruciformis
Figure 10
Figure 10. Clinical photo showing multiple well defined PV-like lesions on the trunk
PV, pityriasis versicolor
See this image and copyright information in PMC

References

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