Skin nonmelanocytic tumor
Lymphoma and related disorders (see also Lymphoma chapter)
Primary cutaneous lymphoma
Author: Christopher S. Hale, M.D.
Topic Completed: 1 July 2012
Minor changes: 15 January 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed search: primary cutaneous [title] lymphoma skin
Cite this page: Hale CS. Primary cutaneous lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticprimarycutaneouslymphoma.html. Accessed March 6th, 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:
- (a) primary cutaneous follicle center lymphoma (PCFCL)
- (b) primary cutaneous marginal zone lymphoma (PCMZL)
- (c) intravascular large B cell lymphoma, diffuse large B cell lymphoma, leg type (DLBCLLT)
- (d) diffuse large cell B cell lymphoma - other;
- 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
Additional references
- J Clin Aesthet Dermatol 2010;3:21, J Clin Pathol 59;8:813, Am J Surg Pathol 2004;28:719
- Mutational analysis (Mod Path 2004;17:623)
