II. Epidemiology
- Cutaneous B-cell Lymphoma (CBCL) Incidence: 4 per Million persons
III. Pathophysiology
- Extranodal Non-Hodgkin Lymphoma
IV. Types: Primary Cutaneous Lymphoma
- Cutaneous B-cell Lymphoma (CBCL, 25% of cases)
- Primary Marginal Zone Lymphoma (PCMZL, 25-30% of CBCL)
- Males (60%) aged 20 to 60 years old
- Red Papules, Plaques or Nodules (often solitary) on trunk and extremities
- Treated with low dose Radiation Therapy, excision , intralesional steroids or observation
- Multiple sites are treated with low dose Radiation Therapy or Rituximab
- Indolent Lymphoma with 99% five year survival (50% relapse rate)
- Primary Cutaneous Follicle Center Lymphoma (PCFCL, 30-60% of CBCL)
- Males (60%) aged 40 to 70 years old
- Red Papules, Plaques or Nodules (often solitary, may be clustered) on head, neck, trunk
- Treated with low dose Radiation Therapy, excision , intralesional steroids or observation
- Multiple sites are treated with low dose Radiation Therapy or Rituximab
- Indolent Lymphoma with 95% five year survival (30% relapse rate)
- Primary Cutaneous Diffuse Large B-Cell Lymphoma (PCDLBCL, 20-40% of CBCL)
- Females (66%) age >60 years
- Red to blue Nodules (often solitary) on the lower leg
- Treated with systemic Chemotherapy and possible Radiation Therapy
- Aggressive Lymphoma with 30-80% five year mortality with R-CHOP (65% relapse rate)
- EBV-Positive Mucocutaneous Ulcer (EBVMCU, rare)
- Age >60 years, associated with Immunosuppression
- Well circumscribed, painful Mucosal Ulcers
- Indolent Lymphoma
- Primary Marginal Zone Lymphoma (PCMZL, 25-30% of CBCL)
- Primary Cutaneous T-Cell Lymphoma (CTCL, 75% of cases)
- See Cutaneous T-Cell Lymphoma
- This topic focuses instead on Cutaneous B-cell Lymphoma