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Published on: October 14, 2021
Acral necrotizing mycosis fungoides
Tasneem Poonawalla1, Dan Jones, Fredrick Hagemeister
1Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Clinical Lymphoma & Myeloma
|October 20, 2005
Summary
A rare, aggressive cutaneous T-cell lymphoma transformed from mycosis fungoides, presenting as necrotizing ulcerative lesions. Despite radiation, the patient succumbed to opportunistic infections within three months.
Area of Science:
- Dermatology
- Oncology
- Infectious Diseases
Background:
- Cutaneous T-cell lymphoma (CTCL) can transform into aggressive subtypes.
- Mycosis fungoides is the most common type of CTCL.
- Necrotizing ulcerative presentations of CTCL are rare and aggressive.
Purpose of the Study:
- To report a case of transformed, necrotizing ulcerative cutaneous T-cell lymphoma.
- To highlight the clinical and pathological features of this aggressive CTCL subtype.
- To discuss the challenges in managing such advanced cases.
Main Methods:
- Case study of a 65-year-old male with a history of acral eczema and mycosis fungoides.
- Clinical examination of necrotic lesions on the toe and lip.
- Histopathological analysis and immunohistochemistry (CD3, CD4, CD8, CD30, CD56, TIA-1).
- Molecular analysis for Epstein-Barr virus (EBV) and T-cell receptor (TCR) gene rearrangement.
Main Results:
- The patient presented with transformed, necrotizing ulcerative CTCL with necrotic eschars.
- Tumor markers were positive for CD3, EBV-encoded small RNA, and TCR gamma gene rearrangement.
- Markers CD4, CD8, CD30, CD56, and TIA-1 were negative.
- Lesions showed opportunistic organism growth.
- Initial response to radiation therapy was observed.
Conclusions:
- Transformed necrotizing ulcerative CTCL is a rapidly progressing and fatal condition.
- Aggressive management and prompt treatment of opportunistic infections are crucial.
- This case underscores the importance of early diagnosis and comprehensive treatment strategies for advanced CTCL.
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