Correctly classifying adnexal neoplasms is a difficult aspect of skin pathology. In some circumstances, designating a lesion merely as "benign adnexal neoplasm" is adequate. However, there are some instances when further classification is essential. In such cases, these benign adnexal neoplasms may serve as a clinical marker for genetic syndromes with serious systemic associations, particularly systemic malignancies. This article discusses these syndromes, specifically Muir-Torre syndrome, Birt-Hogg-Dubé syndrome, and Cowden's syndrome, and their associated adnexal neoplasms.
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