Researchers Report Rare Case of Squamous Cell Carcinoma in Sun-Shielded Area in Epidermodysplasia Verruciformis

A newly published case report in Case Reports in Dermatological Medicine described an unusual presentation of squamous cell carcinoma (SCC) in a patient with epidermodysplasia verruciformis (EV).¹

Unlike the typical pattern of SCC in EV, which predominantly occurs in sun-exposed areas,² this case documented malignant transformation in a consistently covered region of the scalp.

Squamous cell carcinoma on the face | Image Credit: © DermNet

Background and Methods

EV is a rare, inherited disorder that impairs the skin’s immune defense against beta-human papillomavirus (HPV), increasing susceptibility to widespread cutaneous lesions and malignant transformation.² Most cases of SCC in EV develop in sun-exposed areas, highlighting the role of ultraviolet radiation as a key cofactor in oncogenesis.³

Researchers presented the case of a 28-year-old Palestinian woman with a longstanding history of EV presented with multiple painful, ulcerative scalp lesions that had progressively worsened over a year. Despite wearing a hijab and working primarily indoors, ultimately limiting her sun exposure, she developed recurrent malignant lesions requiring surgical intervention.

Findings

The patient had initially been diagnosed with EV as a teenager, with wart-like lesions appearing on her face, scalp, and extremities. She was previously treated with isotretinoin, but her medication was discontinued before her current presentation.

The first suspicious scalp lesion appeared 3 years prior, later confirmed as trichoblastic carcinoma after excision.

Over time, additional nodular lesions emerged, prompting a 6 × 9-cm surgical excision of the right occipital scalp. Histopathology revealed a combination of trichoblastic carcinoma and verrucous carcinoma, both malignancies not typically associated with EV in non-sun-exposed regions.

The case presents a rare departure from the conventional understanding of SCC development in EV. While HPV-driven oncogenesis remains a key factor, the absence of UV exposure suggests other mechanisms, such as genetic predisposition or chronic viral persistence, may contribute to malignant progression in EV patients, according to authors of the case report.

Conclusions

"The rarity of this case underscores its exceptional nature within the context of EV-associated complications," wrote Jebrini et al.

The case challenges the assumption that SCC in EV is exclusively linked to sun exposure, highlighting the need for routine surveillance in all EV patients, regardless of UV exposure history, according to authors. The case also raises important considerations for dermatologists managing EV, particularly regarding long-term treatment strategies and the role of systemic therapies in preventing malignant transformation.

Moving forward, future research may explore additional risk factors that may predispose EV patients to skin cancer in non-sun-exposed regions.

References

1. Jebrini N, Dwaik M, Jaber M, et al. Unusual presentation of epidermodysplasia verruciformis (EV) in non-sun exposed area: a case report. Case Rep Dermatol Med. Published March 26, 2025. doi:10.1155/crdm/7801944
2. Myers DJ, Kwan E, Fillman EP. Epidermodysplasia verruciformis. StatPearls. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. https://www.ncbi.nlm.nih.gov/books/NBK534198/
3. Fania L, Didona D, Di Pietro FR, et al. Cutaneous squamous cell carcinoma: from pathophysiology to novel therapeutic approaches. Biomedicines. 2021;9(2):171. Published 2021 Feb 9. doi:10.3390/biomedicines9020171