Dermatology
B Cell Lymphomas of the Skin
Built from Dermatology, 5th Edition

What’s inside
11 sections · 96 slides
Overview
- What this topic covers
- B cells, lymphoma, and the skin
Background and Classification
How primary cutaneous B cell lymphoma was recognized, how common it is, and how it is classified
- History: skin as a primary site
- How common is cutaneous B cell lymphoma
- Who gets it
- What causes it: infection and immune signals
- The five WHO-EORTC/ICC categories
- Classification of B cell lymphomas with primary cutaneous manifestations – WHO-EORTC 2018 and ICC 2022
- Primary vs. secondary skin lymphoma matters
- Staging work-up for confirmed skin lymphoma
- Recommended staging investigations for patients with a confirmed diagnosis of B cell lymphoma involving the skin
Primary Cutaneous Follicle Center Lymphoma
The most common low-grade B cell lymphoma of the skin
- Primary cutaneous follicle center lymphoma (PCFCL)
- Cutaneous follicle center lymphoma of the eyebrow and forehead
- Crosti's lymphoma: a large back nodule with surrounding erythema
- A second presentation on the forehead
- Ulcerated follicle center lymphoma tumors on the scalp
- Ulcerated follicle center lymphoma on the back (Crosti's pattern)
- PCFCL: outlook
- PCFCL: what the biopsy shows
- Follicle center lymphoma, diffuse type: infiltrate and cell detail
- Follicle center lymphoma, follicular type: neoplastic follicles
- Neoplastic follicles, a second example
- Monomorphous follicles, additional field
- PCFCL: immunostains and genetics
Primary Cutaneous Marginal Zone B Cell Lymphoproliferative Disorder
A low-grade B cell disorder often linked to chronic antigen stimulation, including Borrelia infection
- Primary cutaneous marginal zone B cell lymphoproliferative disorder (PCMZLD)
- PCMZLD: what it looks like
- Marginal zone lymphoproliferative disorder: a firm back nodule
- Two plaques on the foot with confirmed Borrelia DNA in the infiltrate
- Two marginal zone nodules on the shoulder
- Nodule arising within acrodermatitis chronica atrophicans
- PCMZLD and Borrelia infection
- PCMZLD: outlook
- PCMZLD: what the biopsy shows
- Nodular infiltrate with monotypic kappa light chain expression
- Spectrum of cell types seen in PCMZLD
- Monoclonal light chain expression as a diagnostic feature
- Reactive nodules and neoplastic marginal zone cells
- A second example of nodular architecture and light chain restriction
- PCMZLD: immunostains and genetics
Diffuse Large B Cell Lymphoma, Leg Type
The aggressive large-cell lymphoma that favors elderly women's lower legs
- Diffuse large B cell lymphoma, leg type (DLBCLLT)
- DLBCLLT: what it looks like
- Diffuse large B cell lymphoma, leg type: nodules and a flat tumor on both legs
- Multiple red-brown tumors on the lower leg
- Coalescing papulonodules and plaques on the lower leg
- Papulonodules and a tumor on the lower leg, a further example
- DLBCLLT: outlook and the need for staging
- DLBCLLT: what the biopsy shows
- Dense diffuse infiltrate of large round cells
- Large round cells at higher magnification
- DLBCLLT: immunostains that separate it from PCFCL
- Bcl-2 and MUM-1 staining: diffuse PCFCL versus DLBCLLT compared
- Diagnostic approach for cutaneous large B cell lymphomas
- Immunohistochemical marker expression across large B cell lymphomas with skin involvement
- DLBCLLT: genetics and prognostic markers
Intravascular Large B Cell Lymphoma
A rare, aggressive lymphoma that grows inside blood vessels
- Intravascular diffuse large B cell lymphoma (IVDLBCL)
- IVDLBCL: outlook and pathology
- Intravascular large B cell lymphoma: tumor cells within dermal vessels
- Intravascular tumor cells, endothelial staining confirms location
Precursor B Lymphoblastic Lymphoma/Leukemia and Other Rare Types
A childhood-predominant, highly aggressive lymphoma, plus a short list of other rare cutaneous B cell entities
- Precursor B lymphoblastic lymphoma/leukemia
- Precursor B lymphoblastic lymphoma: the "mosaic-stone" cell arrangement
- A large tumor on a child's face
- Precursor B-LBL: confirming the diagnosis
- Other rare cutaneous B cell lymphomas
Telling the Lymphomas Apart
Working through mimics, pseudolymphoma, and secondary skin disease
- General principles of differential diagnosis
- Using immunostains and PCR to sort out cutaneous B cell lymphomas
- Full evaluation pathway, with example PET-CT staging images
- PCFCL vs. Borrelia-induced lymphocytoma cutis
- PCMZLD vs. B cell chronic lymphocytic leukemia and mantle cell lymphoma
- DLBCLLT and IVDLBCL: key look-alikes
- Precursor B-LBL and post-transplant disease
- Post-transplant lymphoproliferative disorders
Treatment
From watchful waiting for indolent disease to systemic chemotherapy for aggressive subtypes
- Choosing a treatment: the general approach
- Local treatment for solitary or few lesions
- Antibiotics, interferon, and rituximab
- Aggressive disease: chemotherapy, CAR-T, and transplant
Plasma Cell Dyscrasias and Multiple Myeloma
From MGUS to myeloma, and the many skin conditions a monoclonal gammopathy can cause
- What is a plasma cell dyscrasia
- Defining symptomatic myeloma
- Skin conditions linked to a monoclonal gammopathy
- Cutaneous conditions associated with monoclonal gammopathy
- Cutaneous conditions associated with monoclonal gammopathy (continued)
- Subcorneal pustular dermatosis with an IgA kappa monoclonal gammopathy
- Detecting the monoclonal protein
- Plasma cell skin infiltrates: pathology
- Follicular spicules in a patient with multiple myeloma
- Cutaneous Waldenstrom macroglobulinemia
Cutaneous and Systemic Plasmacytosis
A reactive, polyclonal plasma cell proliferation most often reported in Asian patients
- Cutaneous and systemic plasmacytosis
- Cutaneous and systemic plasmacytosis: back involvement
- Key takeaways
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Dermatology, 5th Edition (2-Volume Set)