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Desmoplastic trichoepitheliomas (DTEs) are benign cutaneous neoplasms that originate from the hair follicle and exhibit a preference for the facial region. This type of neoplasm is characterized by accelerated growth, with vigorous histologic and immunohistochemical features that may be confused with other skin cancers. Thus, the objective of this study is to establish a definitive diagnosis that can be widely used. This review was systematically carried out and includes case series and studies to establish valuable data that can be used for research. The articles were sought in PubMed, MEDLINE, and Google Scholar using the keywords “desmoplastic trichoepithelioma,” “morphea basal cell carcinoma,” “microcystic adnexal carcinoma,” “syringoma,” and “cutaneous breast carcinoma.” From a total of 65 journal articles, we chose 42 studies describing the clinical features, etiology, histopathology, and immunohistochemical characteristics of tumors. After quality assessment, 10 studies were selected, representing the differentiating features among the four mentioned cutaneous tumors. The differential diagnosis of DTE also includes other cutaneous and follicular tumors. At present, there is no standardized grading system for trichogenic tumors, although several symptomatic terms have been offered. More recently, immunohistochemistry and histopathological studies support the differentiation between the above-mentioned cutaneous tumors. However, additional research needs to be conducted to obtain complete information regarding the specific distinct traits of the indicated cutaneous tumors.

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Desmoplastic Trichoepithelioma: Histopathologic and Immunohistochemical Criteria for Differentiation of a Rare Benign Hair Follicle Tumor From Other Cutaneous Adnexal Tumors