Non-Melanoma Skin Cancers (NMSCs) are the most common skin neoplasms, and they are mainly divided into basal cell carcinomas (BCC) and cutaneous squamous cell carcinomas (cSCC).
Hereditary epidermolysis bullosa (EB) is a rare genodermatosis characterized by skin fragility and blistering of the skin and mucous membranes, in reaction to minimal trauma.
EB is genetically and clinically very heterogeneous and consists of four main types: simplex EB, junctional EB, dystrophic EB, and Kindler EB. Based on the clinical characteristics, blister distribution, and the severity of clinical signs, it is possible to identify several EB subtypes.
Each type has numerous complications, the most common one being the development of NMSCs. BCCs are commonly found in severe simplex EB, cSCC is the prevalent NMSC for junctional and dystrophic EB patients.
Surgical excision of tumors is generally the standard procedure for NMSCs. Wound closure in EB patients is often pursued with autologous split skin, epidermal or full-thickness grafting, dermal substitutes, and/or secondary intention healing.
AIM OF THE WORK
The aim of our work was to analyze the postoperative outcomes of cSCCs excised from EB patients in our clinic, in order to have a better knowledge of the main critical issues with this cohort of patients in their surgical management and oncological follow-up.
MATERIALS AND METHODS
We collected data from a cohort of 5 patients diagnosed with cSCC, treated at Modena University Hospital from February 2008 to May 2021. Collected data include patients’ age and sex, date of cSCC diagnosis, relapses, site of the neoplasia, number of surgical interventions needed for radical excision, use of dermal substitutes, postoperative infections.
The results of the data analysis showed the tendency of EB patients to develop numerous cSCCs that often tended to relapse after complete excision with clear margins. Moreover, multiple surgeries were commonly necessary to achieve radical excision of cSCCs with clear margins. A great part of the studied population used dermal substitutes, the most commonly used being Matriderm®. However, the postoperative infection rates were higher when the dermal substitutes were used.
In conclusion, we observed a particularly aggressive progress of cSCC in EB patients. Our data suggest that the best approach for these tumors is the early diagnosis and a strict follow-up after surgery.