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An 80-year-old man had a persistent temple ulcer initially diagnosed as AK. Repeat deep biopsy demonstrated coexisting DFSP and bowenoid AK, confirmed by immunohistochemistry showing strong CD34 expression. Mohs micrographic surgery achieved clear margins, underscoring clinicopathological correlation and repeat biopsy for complex, non-healing skin lesions. Management requires complete surgical excision with sufficient margins.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/collision-of-actinic-keratosis-and-dermatofibrosarcoma-protuberans-within-a-single-lesion-as-a-diagnostic-pitfall-a-case-report/448317/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/collision-of-actinic-keratosis-and-dermatofibrosarcoma-protuberans-within-a-single-lesion-as-a-diagnostic-pitfall-a-case-report/448317.png","ImageObject",300,407,{"name":92,"@type":93},"Genevieve","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-31","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":44},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"Why can this case be a diagnostic pitfall?","Question",{"text":112,"@type":113},"The initial punch biopsy captured only the epidermal component and led to an AK diagnosis. Coexisting DFSP became apparent only after repeat deep biopsy because DFSP originates in the dermis.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How was DFSP confirmed in the repeat biopsy?",{"text":117,"@type":113},"Immunohistochemistry confirmed DFSP with strong CD34 expression, supporting coexistence with bowenoid AK.",{"name":119,"@type":110,"acceptedAnswer":120},"What treatment achieved complete management in this case?",{"text":121,"@type":113},"The lesion was excised using Mohs micrographic surgery to obtain histologically clear margins, followed by reconstruction with a parotid fascia-based bilobed fasciocutaneous flap.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},448317,1791080117,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":44,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":24,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144,"preview_page_count":4},1374391974585,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","Archives of Craniofacial Surgery  \nArch Craniofac Surg Vol.26 No.6, 261-265 [https://doi.org/10.7181/acfs.2025.0068](https://doi.org/10.7181/acfs.2025.0068)  \nCollision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report  \nYujin Ahn, Joon Ho Lee  \nDepartment of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, Gyeongju, Korea  \nDermatofibrosarcoma protuberans (DFSP) is a rare locally aggressive cutaneous sarcoma, whereas actinic keratosis (AK) is a common premalignant epidermal lesion. The coexistence of these two entities within a single facial lesion is extremely rare and may complicate diagnosis, particularly when an initial biopsy captures only the epidermal component. We present the case of an 80-year-old man with a persistent facial lesion initially diagnosed as AK on punch biopsy. Owing to the atypical clinical features, a repeat deep biopsy revealed the coexistence of DFSP and bowenoid AK. Immunohistochemistry confirmed the presence of DFSP with strong CD34 expression. The lesion was excised using Mohs micrographic surgery to achieve clear margins. This case highlights the importance of clinicopathological correlation and repeat biopsy for the accurate diagnosis of complex skin lesions. Surgical excision with sufficient margins is the key for optimal management.  \nAbbreviations: AK, actinic keratosis; DFSP, dermatofibrosarcoma protuberans; MMS, Mohs micrographic surgery; SMA, smooth muscle actin  \nKeywords: Actinic keratosis / Case reports / Comorbidity / Dermatofibrosarcoma / Mohs surgery / Skin neoplasms  \nINTRODUCTION  \nDermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive, cutaneous soft tissue sarcoma originating in the dermis [1]. It accounts for less than 0.1% of all malignancies and about 1% of soft tissue sarcomas [2]. DFSP most commonly arises on the trunk and extremities, but its occurrence on the face is extremely uncommon [3]. In contrast, actinic keratosis (AK) is the most common premalignant epidermal lesion  \nCorrespondence: Joon Ho Lee  \nDepartment of Plastic and Reconstructive Surgery, Dongguk University College of Medicine, 123 Dongdae-ro, Gyeongju 38066, Korea  \n[E-mail: urrussa@naver.com](E-mail: urrussa@naver.com)  \nHow to cite this article:  \nAhn Y, Lee JH. Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall: a case report. Arch Craniofac Surg 2025;26(6):261-265. [https://doi.org/10.7181/acfs.2025.0068](https://doi.org/10.7181/acfs.2025.0068)  \nReceived October 20, 2025 / Revised November 18, 2025 / Accepted December 6, 2025  \nworldwide and typically develops in chronically sun-exposed areas, such as the face [4]. There is no known association between DFSP and AK, and their pathogenesis differs fundamentally. Histologically, DFSP arises in the dermis, whereas AK is confined to the epidermis. Therefore, coexistence of these lesions at the same anatomical site is extremely rare. This manuscript was written to report a rare case of concurrent AK and DFSP arising within a single facial lesion, highlighting the diagnostic challenges when superficial biopsy only reveals the epidermal component. Through this case, we emphasize the need for repeat or deeper biopsies in atypical or non-healing lesions to avoid misdiagnosis and ensure appropriate management.  \nCASE REPORT  \nAn 80-year-old man with underlying hypertension and diabetes mellitus presented to the dermatology department with the  \n© 2025 Korean Cleft Palate-CraniofacialAssociation  \nThis is an OpenAccess article distributed under the terms of the Creative CommonsAttribution Non-Commercial License ([https://creativecommons.org/](https://creativecommons.org/)  \n[licenses/by-nc/4.0/](licenses/by-nc/4.0/)) [which permits unrestricted non-commercial use](which permits unrestricted non-commercial use), [distribution](distribution), [and reproduction in any medium](and reproduction in","cbCaithSU9wNftZI","https://ap.wps.com/l/cbCaithSU9wNftZI","pdf",891733,"English","# Introduction\n# Case report\n## Clinical presentation\n## Repeat biopsy and diagnosis\n## Treatment and reconstruction\n# Discussion","[{\"question\":\"Why can this case be a diagnostic pitfall?\",\"answer\":\"The initial punch biopsy captured only the epidermal component and led to an AK diagnosis. Coexisting DFSP became apparent only after repeat deep biopsy because DFSP originates in the dermis.\"},{\"question\":\"How was DFSP confirmed in the repeat biopsy?\",\"answer\":\"Immunohistochemistry confirmed DFSP with strong CD34 expression, supporting coexistence with bowenoid AK.\"},{\"question\":\"What treatment achieved complete management in this case?\",\"answer\":\"The lesion was excised using Mohs micrographic surgery to obtain histologically clear margins, followed by reconstruction with a parotid fascia-based bilobed fasciocutaneous flap.\"}]","Collision of actinic keratosis and dermatofibrosarcoma protuberans within a single lesion as a diagnostic pitfall - a case report | PDF",1790726542,13]