Lymph

Cutaneous B-cell Lymphoma

Cutaneous B-cell Lymphoma, CBCL, Primary Cutaneous Follicle Center Lymphoma, PCFCL, Primary Cutaneous Diffuse Large B-Cell Lymphoma, PCDLBCL, Primary Cutaneous Marginal Zone Lymphoma, PCMZL

See Also

Epidemiology

Cutaneous B-cell Lymphoma (CBCL) Incidence: 4 per Million persons

Pathophysiology

Extranodal Non-Hodgkin Lymphoma

Types
Primary Cutaneous Lymphoma

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

References