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Published on: March 30, 2018
Primary cutaneous marginal-zone lymphoma
Joshua Farhadian1, Vtaly Terushkin, Shane A Meehan
1Ronald O. Perelman Department of Dermatology, NYU School of Medicine, NYU Langone Medical Center.
Insights
Primary cutaneous marginal-zone lymphoma (PCMZL) is a common skin cancer with a high survival rate. This case highlights a rare presentation of PCMZL in a young woman.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Primary cutaneous B cell lymphomas (PCBCL) are the second most common cutaneous lymphomas.
- Key subtypes include primary cutaneous marginal-zone lymphoma (PCMZL), primary cutaneous follicle-center lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg type.
- PCMZL is characterized by an indolent course and a five-year survival rate nearing 99%.
Purpose of the Study:
- To report a case of primary cutaneous marginal-zone lymphoma (PCMZL) in a young Hispanic woman.
- To describe an unusual presentation of PCMZL on the face.
Main Methods:
- Case report of a 33-year-old Hispanic woman.
- Clinical presentation: firm, pruritic, pink papules on the forehead and cheeks.
- Diagnosis of PCMZL confirmed.
Main Results:
- The patient presented with facial papules, an atypical location for PCMZL.
- PCMZL lesions commonly appear as erythematous-to-violaceous papules, plaques, or nodules, typically on the trunk or arms.
- Approximately 50% of patients have solitary skin lesions.
Conclusions:
- Primary cutaneous marginal-zone lymphoma (PCMZL) can present atypically in terms of patient demographics and lesion location.
- Early diagnosis and appropriate treatment are crucial for managing PCBCL.
- This case underscores the importance of considering PCMZL in the differential diagnosis of facial papular lesions.
Abstract:
Primary cutaneous B cell lymphomas (PCBCL) are thesecond most common type of primary cutaneouslymphoma. The three main types of PCBCL areprimary cutaneous marginal-zone lymphoma(PCMZL), primary cutaneous follicle-centerlymphoma, and primary cutaneous diffuse largeB-cell lymphoma, leg type. PCMZL has an indolentcourse with a five-year survival rate approaching99%. Lesions most often present on the trunk or armas erythematous-to-violaceous papules, plaques, ornodules. Approximately one-half of patients havesolitary skin lesions. Treatment options includesurgery, radiation, and topical, intralesional orsystemic therapy. We present the case of a 33-yearoldHispanic woman with firm, pruritic, pink papuleson the forehead and cheeks, who was diagnosedwith PCMZL.
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