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Extramammary Paget disease (EMPD) is a rare skin cancer on apocrine gland areas. Early diagnosis and surgical excision offer a favorable prognosis for this condition.

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Area of Science:

  • Dermatology
  • Oncology
  • Pathology

Background:

  • Paget disease of the nipple (mammary Paget disease) was first described in 1874.
  • Extramammary Paget disease (EMPD) was identified in 1889 on the scrotum and penis.
  • EMPD shares epidermal Paget cells and clinical presentation with mammary Paget disease but differs in location and histogenesis.

Purpose of the Study:

  • To provide a comprehensive review of extramammary Paget disease (EMPD).
  • To cover the history, epidemiology, pathogenesis, clinical presentation, diagnosis, and management of EMPD.
  • To discuss recent advances and new observations regarding EMPD.

Main Methods:

  • Review of existing literature on extramammary Paget disease (EMPD).
  • Discussion of diagnostic tools including immunohistochemical staining.
  • Analysis of treatment strategies, focusing on surgical excision.

Main Results:

  • EMPD presents as an erythematous plaque on apocrine gland-bearing areas in older individuals.
  • Immunohistochemistry is crucial for differentiating primary EMPD from secondary EMPD and other conditions.
  • Surgical excision is the primary treatment, often leading to favorable outcomes.

Conclusions:

  • A thorough patient history and physical examination are essential due to the potential for underlying malignancy.
  • Recent research explores chemokine receptor expression for prognostic insights.
  • Understanding EMPD's multifaceted aspects is key for effective patient management.