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From Morphea to Dermatofibrosarcoma Protuberans.

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This case report details a rare instance of dermatofibrosarcoma protuberans (DFSP) developing within a morphea profunda (MP) plaque. Repeated biopsies may have triggered DFSP development in the MP lesion, highlighting diagnostic and surgical challenges.

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Area of Science:

  • Dermatology and Oncology
  • Autoimmune diseases and soft tissue neoplasms

Background:

  • Morphea profunda (MP) is a subtype of localized scleroderma causing discomfort and impaired skin motility.
  • Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue neoplasm with high recurrence rates but low metastatic potential.

Observation:

  • A 39-year-old female presented with a 15-year history of a slow-growing abdominal lesion initially diagnosed as MP.
  • The lesion evolved to include palpable nodules, and subsequent biopsy confirmed DFSP.
  • The patient had undergone three previous biopsies for the lesion.

Findings:

  • Histopathology and CD34 immunohistochemistry confirmed DFSP arising within a morphea profunda plaque.
  • Complete tumor excision was performed, followed by re-excision due to narrow margins.
  • No disease progression or recurrence was noted during one year of follow-up.

Implications:

  • This is the first reported case of DFSP developing within an MP plaque, suggesting potential iatrogenic triggers like repeated biopsies.
  • DFSP diagnosis can be delayed due to slow growth and non-specific initial findings, necessitating thorough histopathological evaluation.
  • Achieving wide surgical margins is critical for managing DFSP and preventing recurrence, underscoring the importance of surgical expertise and follow-up.