Acta Dermato-Venereologica issue 50:1 98-1CompleteContent | Page 34

132 SHORT COMMUNICATION Multiple Basal Cell Carcinoma Arising in a Verrucous Epidermal Naevus: Clinical, Histological and Therapeutic Observations Francesca SATOLLI, Miriam ROVESTI, Maria Beatrice DE FELICI, Alfredo ZUCCHI, Calogero PAGLIARELLO and Claudio FELICIANI Department of Dermatology, University of Parma, via Gramsci 14, IT-43100 Parma, Italy. E-mail. [email protected] Accepted Jul 24, 2017; Epub ahead of print Jul 24, 2017 Basal cell carcinoma (BCC) is the most common malig- nancy in humans and it usually occurs as a solitary lesion on sun-exposed areas. Its association with non-organoid epidermal naevi (NONEEN) is an uncommon event. To our knowledge, there have been very few cases reported in the literature so far, and, among these, only two cases of multiple BCCs arising in a verrucous epidermal naevi (1, 2). We report a case of a 73-year-old woman affected by multiple BCCs that have arisen within the context of a congenital verrucous epidermal naevus of the left thigh and left foot. We investigated the possible role of genetic mutations. The challenge was also to find an effective treatment for BCCs, considering the multiplicity of lesions. There­ fore, we also used some off-label treatments, such as ingenol mebutate, which resulted in partial clearance. During follow-up, the patient also developed a superficial melanoma on the same leg as the verrucous epider- mal naevus. Molecular examination with PCR and DNA sequencing revealed no pathological mutations in the PTCH1 gene. However, a different mutation located in the 5 th untranslated region (UTR) of PTCH-1 was found and considered. During the last 5 years of follow-up, the patient developed 47 BCCs which occurred in a linear naevus. Moreover, she develo- ped a superficial melanoma IA (according to AJCC 2009 staging guidelines for melanoma (3) Fig. S2 1 ) on the same leg that was treated surgically (by excisional biopsy and wide excision). At each subsequent visit, both surgery and photodynamic therapy using methyl aminolevulinate (MAL-PDT) were offered to the patient to remove the remaining lesions but she deter- minedly refused due to the pain and suffering caused by these procedures. We therefore used imiquimod 5% cream once a day, https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-2753 1 CASE REPORT A Caucasian woman in her seventies was referred to our department because of the re- cent appearance of about 30 pinkish-purple or pigmented papulo-nodular lesions within the context of a pre-existing congenital ver- rucous epidermal naevus of the left leg (Fig. 1a, b). Clinical and dermoscopical examina- tions (Fig. 1e) revealed features of BCCs. We decided to surgically remove the 4 lar- gest lesions (Fig. 1c, d). Histopatho­logical ex- amination showed acanthosis, papillomatosis and hyperkeratosis, typical of non-organoid non-epidermolytic epidermal naevi and, in this context, also nests of basaloid cells with a palisade pattern and hyperchromatic nuclei diagnostic of BCCs (Fig. S1a 1 ). Immuno- histochemistry with BerEP4 confirmed the diagnosis of BCCs (Fig. S1b 1 ). Although the patient had neither the clinical features of a naevoid basal cell carcinoma syndrome (such as palmar pits, keratocystic odontogenic tumours, ske- letal anomalies, ectopic calcifications or macrocephaly) nor a family history for the syndrome, we decided to investigate the PTCH1 gene to exclude the diagnosis of mosaicism in Gorlin syndrome. doi: 10.2340/00015555-2753 Acta Derm Venereol 2018; 98: 132–133 Fig. 1. Clinical characteristics. The congenital verrucous epidermal naevus (a, b). The surgically excised BCCs arising within the nevus’ (c, d). Dermatoscopic evaluation of BCCs showed in detail large blue-grey ovoid nests or blotches, structureless of leaf-like areas (e). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.