Skin nonmelanocytic tumor

Lymphoma and related disorders (see also Lymphoma chapter)

Primary cutaneous lymphoma

Topic Completed: 1 July 2012

Minor changes: 16 April 2021

Copyright: 2003-2021,, Inc.

PubMed search: primary cutaneous [title] lymphoma skin

Christopher S. Hale, M.D.
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Cite this page: Hale CS. Primary cutaneous lymphoma. website. Accessed December 7th, 2021.
Primary cutaneous B cell lymphoma
  • < 20% of primary cutaneous lymphomas
  • By definition, no evidence of extracutaneous disease is identified for 6 months after initial diagnosis
  • Marginal zone lymphomas often associated with Borrelia burgdorferi infection

  • Similar morphology to nodal disease, but more indolent Subtypes include:
  • Pan B cell markers are CD20, CD79a (J Clin Aesthet Dermatol 2010;3:21)

  • Primary Cutaneous Follicle Center Lymphoma:
    • Positive for CD20 and CD79a with follicular pattern
    • Follicular and diffuse patterns of PCFCL typically BCL6 positive
    • CD10 expressed in follicular pattern and typically negative in diffuse pattern
    • BCL2, CD5, and CD43 negative
    • BCL2 positivity suggests nodal origin with skin involvement
    • t(14;18) is rare in cutaneous follicular lymphoma, but is characteristic of nodal follicular lymphoma

  • Primary Cutaneous Marginal Zone Lymphoma:
    • Positive for CD20, CD79a, and BCL2; negative staining for CD5, CD10, CD43, BCL6

  • Diffuse Large B cell Lymphoma, leg type:
    • BCL2 positive (negative staining suggests diffuse large B cell lymphoma, other)
    • Positive for CD20, CD79a, MUM1 / IRF4 and usually BCL6 or CD10
Subcutaneous, blastic NK, NK / T cell or other cytotoxic T cell lymphoma
  • These cases include patients with skin and non skin disease at diagnosis, as well as skin only
  • Excludes mycosis fungoides and peripheral T cell lymphoma
  • All CD30 negative with medium / large cells or subcutaneous panniculitis-like

  • (a) Subcutaneous panniculitis-like T cell lymphoma:
    • Indurated, erythematous and discolored plaques on extremities
    • Alpha / beta CD8+ cytotoxic T cells, with almost exclusive involvement of subcutaneous tissue resembling lobular panniculitis
    • Recommend to exclude cases with epidermal involvement from this category
    • Tumor cells have pleomorphic nuclei and adipocyte rimming (not specific for this diagnosis)
    • CD3+, CD8+, TIA1+, EBV-, estimated 5 year survival is 80% with systemic steroid therapy

  • (b) Blastic NK cell lymphoma:
    • Usually multiple bruise-like deep red plaques / tumors
    • Involves dermis and surrounding adnexa, grenz zone present, subcutis but no epidermal involvement
    • Monomorphous medium sized cells with fine chromatin resembling blasts of acute myelogenous leukemia (although AML is CD56-)
    • May actually derive from common myeloid and NK precursor called plasmacytoid type 2 dendritic cell
    • CD3-, CD4+, CD8-, CD56+, TIA1-, TdT variable, EBV-
    • Estimated 5 year survival is 0%
    • Eventually become leukemia

  • (c) NK / T cell lymphoma, nasal type:
    • Multiple patches, plaques or nodules
    • May involve epidermis, dermis or subcutis
    • Medium - large pleomorphic or blastic nuclei
    • CD3epsilon+, TIA1+, EBV+
    • Estimated 5 year survival is 0%

  • (d) Epidermotrophic CD8+ T cell lymphoma:
    • Multiple plaques and tumors, similar to disseminated pagetoid reticulosis
    • Ulceration common
    • Alpha / beta negative, CD8+ cytotoxic T cells, with predominant involvement of epidermis, also dermis, adnexae and subcutis
    • CD3+, CD8+; TIA1+, betaF1+; EBV-
    • Estimated 5 year survival is 0%
    • Must rule out mycosis fungoides

  • (e) Cutaneous gamma / delta T cell lymphoma:
    • Multiple plaques and tumors, similar to disseminated pagetoid reticulosis
    • Epidermal involvement with necrosis, interface dermatitis, adnexal involvement, gamma / delta T cells
    • CD4-, CD8-, CD56+, TIA1+, EBV-, estimated 5 year survival is 0%

  • (f) Cutaneous alpha / beta pleomorphic T cell lymphoma:
    • Solitary or multiple plaques and tumors
    • Often epidermal necrosis and adnexal involvement
    • Alpha / beta T helper cells (CD8-) with expression of cytotoxic markers betaF1 and TIA1, different from subcutaneous panniculitis-like T cell lymphoma and epidermotrophic CD8+ T cell lymphoma
    • Estimated 5 year survival is 0%

  • (g) Cutaneous medium / large pleomorphic T cell lymphoma, not otherwise specified:
    • Multiple plaques and tumors, don’t fit other categories
Clinical images

Images hosted on other servers:

Small or medium pleo-
morphic T cell lymphoma
with solitary reddish
nodule on the cheek

Microscopic (histologic) images

Images hosted on other servers:

Cutaneous CD8 epidermotropic T cell lymphoma

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