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. 2022 Oct;61(10):1171-1174.
doi: 10.1111/ijd.16157. Epub 2022 Mar 22.

Surgical management and oncological follow-up of cutaneous squamous cell carcinomas arising in epidermolysis bullosa patients

Alessia Paganelli  1   2 , Erminia Giordano  1 , Chiara Fiorentini  1 , Barbara Ferrari  1 , Camilla Reggiani  1   2 , Federico Garbarino  1   2 , Cristina Magnoni  1
Affiliations

Surgical management and oncological follow-up of cutaneous squamous cell carcinomas arising in epidermolysis bullosa patients

Alessia Paganelli et al. Int J Dermatol. 2022 Oct.

Abstract

Background: Hereditary epidermolysis bullosa (EB) is a rare genodermatosis characterized by skin fragility and blistering of the skin and mucous membranes in reaction to minimal traumas. The development of cutaneous squamous cell carcinomas (cSCCs) is one of the most common medical complications in junctional and dystrophic forms of the disease. Complete surgical excision of cutaneous tumors represents the gold standard of treatment. However, not only recognition of cSCCs can be challenging in the affected skin but also wound closure after surgical excision poses a great therapeutic challenge in EB patients. The aim of our study was to analyze the postoperative outcomes of such patients in order to have a better knowledge of the main critical issues in their surgical management and oncological follow-up.

Methods: We retrospectively identified a cohort of five EB patients treated at Modena University Hospital. Collected data included patient age and sex, date of cSCC diagnosis, relapses/recurrences, site of the neoplasm, number of surgical interventions, use of dermal substitutes, and postoperative infections.

Results: A total of 26 cSCCs were detected in our cohort. Forty-one surgical interventions were necessary to achieve excision of cSCCs with clear margins, varying from 1 to 4 surgical sessions per cSCC. Dermal substitutes were used in most cases but carried a higher infectious risk.

Conclusions: EB patients tend to develop numerous cSCCs that often relapse even after complete excision with clear margins. These results stress the importance of early cSCC diagnosis and strict postsurgical follow-up.

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Figures

Figure 1
Figure 1
Clinical pictures of cSCCs in DEB (Panel a) and JEB (Panel b) patients [Colour figure can be viewed at wileyonlinelibrary.com]
Figure 2
Figure 2
Kaplan–Meier curve of first cSCC diagnosis. The y‐axis reports the total of EB patients. Patient age is represented on the x‐axis [Colour figure can be viewed at wileyonlinelibrary.com]
Figure 3
Figure 3
cSCCs in EB patients: primary tumors and recurrences [Colour figure can be viewed at wileyonlinelibrary.com]
See this image and copyright information in PMC

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