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Mohs excision of multiple basal cell carcinomas in a child with xeroderma pigmentosum: The first case of its kind in the UK: Association of Surgeons in Training 2018 Conference

Research output: Contribution to journalMeeting abstract

K. Aloni, E. Craythorne, K. Johal, H. Fassihi, A. Greig

Original languageEnglish
Pages (from-to)S95
JournalInternational Journal Of Surgery
Volume55
Early online date2 Aug 2018
DOIs
E-pub ahead of print2 Aug 2018
Published2018

King's Authors

Abstract

Introduction: Xeroderma pigmentosum is a rare autosomal recessive condition affecting repair of DNA damaged by exposure to ultraviolet radiation (UVR). The most common feature of the 8 subtypes of the disease is a greatly increased incidence of skin cancers. Despite rigorous photoprotection, patients frequently develop tumours, which can be managed medically, or excised surgically.

Presentation of case: An 8-year-old female with xeroderma pigmentosum complementation group C was referred to the paediatric plastic surgery clinic with multiple basal cell carcinomas (BCCs) on her face. 8 lesions were excised via Mohs micrographic surgery with full thickness skin grafting, harvested from behind her right ear.

Discussion: This case highlights the complexities of managing xeroderma pigmentosum in young children, particularly in a surgical setting. Even with meticulous protection from UVR, patients inevitably develop skin cancers. Topical imiquimod can help treat background actinic damage, superficial and some nodular BCCs, but morphoeic subtypes and previously incompletely excised BCCs are best treated with Mohs micrographic surgery.

Conclusion: Mohs excision allows the full excision of BCCs in paediatric patients with xeroderma pigmentosum, thereby reducing the need for further intervention in the future. In turn, patients are afforded a less interrupted childhood, provided they remain protected from UVR.

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