Dermatology: Practical and Conceptual Image Letter | Dermatol Pract Concept. 2024;14(3):e2024195 1 Perianal Basal Cell Carcinoma: A Diagnostic Challenge Silvia Giordano1, Francesc Alamon-Reig2, Mar Luque Luna2, Paola Castillo3, Agustí Toll4, Cristina Carrera2,5 1 Dermatology Clinic, Department of Medical Science, University of Turin, Turin, Italy 2 Department of Dermatology, University of Barcelona, Hospital Clinic of Barcelona, Barcelona, Spain 3 Departments of Pathology, Hospital Clinic of Barcelona, Barcelona, Spain 4 Department of Dermatology, Hospital del Mar, Universitat Autònoma de Barcelona (UAB), Barcelona, Spain 5 Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto Carlos III, Barcelona, Spain Citation: Giordano S, Alamon-Reig F, Luna ML, Castillo P, Toll A, Carrera C. Perianal Basal Cell Carcinoma: A Diagnostic Challenge. Dermatol Pract Concept. 2024;14(3):e2024195. DOI: https://doi.org/10.5826/dpc.1403a195 Accepted: March 3, 2024; Published: July 2024 Copyright: © Giordano et al. This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Corresponding Author: Silvia Giordano, Dermatology Clinic, Department of Medical Science, University of Turin, Turin, Italy. Email: si.giordano@unito.it Case Presentation A 70-year-old woman with a history of uterine prolapse presented with an asymptomatic ulcerated dark plaque with mucocutaneous involvement in the anorectal region (Figure 1A). Dermoscopically, the lesion exhibited globular blue-black areas with atypical vessels, blue-grey dots, and white structureless areas (Figure 1B). Melanoma was first suspected; however, the biopsy revealed an infiltrating pig- mented basal cell carcinoma (BCC) (Figure 1C). The MRI examination revealed a mucosal involvement with extension up to the internal sphincter (Figure 1D). At the multidisci- plinary tumor board, oral hedgehog inhibitor therapy with vismodegib was initiated, with remarkable shrinkage after three months and good tolerance, thus avoiding mutilating surgery. Teaching Point BCCs in non-sun-exposed areas are exceedingly rare, the anorectal region being the rarest site, comprising less than 0.2% of all BCC cases. This rarity, combined with its ten- dency to present atypically, often makes diagnosis challeng- ing as it can mimic benign conditions but also malignant tumors like pigmented squamous cell carcinoma or mela- noma, as they are more frequent than BCC in this location. 2 Image Letter | Dermatol Pract Concept. 2024;14(3):e2024195 References 1. Tsai TY, Liao CK, Zhang BY et al. Perianal Basal Cell Carcinoma- A Systematic Review and Meta-Analysis of Real-World Data. Diagnostics (Basel). 2023 May 8;13(9):1650. doi: 10.3390/ diagnostics13091650. PMID: 37175041; PMCID: PMC10178443. 2. Gibson GE, Ahmed I. Perianal and genital basal cell carcinoma: A clinicopathologic review of 51 cases. J Am Acad Dermatol. 2001 Jul;45(1):68-71. doi: 10.1067/mjd.2001.114588. PMID: 11423837. Figure 1. (A) Clinical and (B) dermoscopic images of the perianal lesion. (C) Infiltrated basal cell carcinoma. (D) MRI shows mucosal involvement up to the internal sphincter.