Introduction: Primary dermal melanoma (PDM) is a rare and poorly understood variant of cutaneous melanoma but is distinct in its behavior. The exact mechanisms underlying PDM remain uncertain and there is no standardized approach for diagnosis, management, or staging. This review summarizes the current theories on the pathophysiology, diagnostic criteria, and treatment of PDM. Objective: To complete a narrative review about PDM and its current understanding of pathophysiology, clinical presentations, immunohistochemical (IHC) analysis and management. Methods: A comprehensive review of the literature on PubMed, Web Of Science and Embase was conducted to analyze studies on PDM. Studies from the 10 years were included in analysis. Results: PDM shares clinical similarities to melanoma. While wide local excision and sentinel lymph node biopsy remain the standard of care in management, exact diagnostic protocols, staging criteria, and treatment algorithms are not standardized. Medical therapies remain inconclusive regarding efficacy due to a lack of robust data and clinical trials specific to PDM. Identification of IHC markers unique to PDM is crucial for accurate diagnosis, as currently defined markers overlap with those of melanoma. Despite this, PDM’s consistently show a favorable prognosis compared to melanoma, suggesting that they may be a unique clinical entity. Conclusion: PDM remains an enigmatic condition and more research is needed to fully understand its pathophysiology and refine diagnostic and treatment protocols. Standardized diagnostic criteria and management strategies will be essential for improving patient outcomes and aiding clinicians in effectively diagnosing and treating this rare condition.
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Mahmood et al. (2026) studied this question.
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