Lymphoma & related disorders
Mature T/NK cell disorders
Cutaneous / soft tissue involvement
Primary cutaneous CD8+ aggressive epidermotropic T cell lymphoma


Topic Completed: 7 August 2020

Minor changes: 24 August 2020

Copyright: 2001-2020, PathologyOutlines.com, Inc.

PubMed search: Epidermotropic CD8+ lymphoma

Mario L. Marques-Piubelli, M.D.
Roberto N. Miranda, M.D.
Page views in 2019: 527
Page views in 2020 to date: 603
Cite this page: Marques-Piubelli ML, Ferrufino-Schmidt MC, Miranda RN. Primary cutaneous CD8+ aggressive epidermotropic T cell lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBCD8epidermotropic.html. Accessed September 30th, 2020.
Definition / general
Essential features
ICD coding
  • ICD-O: 9709/3 - Cutaneous T cell lymphoma, NOS
Sites
Clinical features
Diagnosis
  • Combination of clinical, histopathological and immunohistochemical features (J Am Acad Dermatol 2012;67:748)
  • Diagnostic criteria (J Am Acad Dermatol 2012;67:748)
    • Constant features
      • Aggressive clinical course with few weeks evolution
      • Widespread ulcerated papules, plaques and tumors without precursor lesion
      • Epidermotropism and nodular or diffuse dermal pleomorphic T cell infiltrate
      • CD8+ / CD4-
    • Variable features
      • Pyoderma gangrenosum-like, pagetoid reticulosis-like and annular erythematous scaling patches
      • Spongiosis, blisters and necrosis
      • Deep lymphoid infiltrate, angiocentrism and angiodestruction
      • Variable expression of CD2, CD7, CD15, CD45 and CD56
Prognostic factors
Case reports
  • 16 year old boy with primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma with cerebral infiltration and treated with cord blood stem cell transplant (SCT) (Intern Med 2018;57:2051)
  • 19 year old woman with primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma simulating pyoderma gangrenosum (Clin Exp Dermatol 2009;34:e261)
  • 29 year old woman with primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma and homozygous mutation in SAMHD1 (JAAD Case Rep 2015;1:227)
  • 47 year old man with primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma and complete durable remission after autologous and allogeneic hematopoietic stem cell transplantation (JAAD Case Rep 2017;3:196)
  • 61 year old man with primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma and history of psoriasis (Ann Dermatol 2018;30:255)
  • 68 year old man with lupus-like initial clinical presentation (Indian Dermatol Online J 2019;10:298)
  • 72 year old man with primary cutaneous aggressive epidermotropic CD8+ cytotoxic T cell lymphoma of the ear (J Laryngol Otol 2007;121:503)
Treatment
  • No definite successful therapy available
  • Multiagent chemotherapy: CHOP (cyclophosphamide, hydroxydorubicin, vincristine and prednisone), CHOEP (cyclophosphamide, hydroxydorubicin, vincristine, etoposide and prednisone), hyper-CVAD (hyper-cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate and cytarabine) and MACOP-B (methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone and bleomycin) (J Am Acad Dermatol 2012;67:748, Mod Pathol 2017;30:761)
    • Unsatisfactory and usually partial response
  • Total skin electron beam therapy with or without oral retinoid (e.g. bexarotene) (J Am Acad Dermatol 2012;67:748, J Am Acad Dermatol 2010;62:300)
    • Partial response and some cases with complete clearance
  • Allogeneic or autologous stem cell transplant (SCT) (J Am Acad Dermatol 2012;67:748, Mod Pathol 2017;30:761, J Am Acad Dermatol 2010;62:300)
    • Setting of unresponsive or relapse after multiagent chemotherapy or early use of combined intensive chemotherapy and SCT
Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Roberto N. Miranda, M.D.
Skin epidermotropism and dermal infiltrate

Skin epidermotropism and dermal infiltrate

Epidermotropism with intraepidermal cluster

Epidermotropism with intraepidermal cluster

Lymphoma at the dermoepidermal junction

Lymphoma at the dermoepidermal junction

Large pleomorphic cells

Large pleomorphic cells

CD3 positivity

CD3 positivity

CD4 negativity of epidermotropic cells

CD4 negativity of epidermotropic cells


CD7 positivity of epidermotropic cells

CD7 positivity of epidermotropic cells

CD8 positivity of epidermotropic cells

CD8 positivity of epidermotropic cells

TIA1 positivity of lymphoma cells

TIA1 positivity of lymphoma cells

Proliferation marker Ki67

Proliferation marker Ki67

Pagetoid reticulosis

Pagetoid reticulosis

Microabscess of Pautrier in pagetoid reticulosis

Microabscess of Pautrier in pagetoid reticulosis


CD3 in pagetoid reticulosis

CD3 in pagetoid reticulosis

CD8 in pagetoid reticulosis

CD8 in pagetoid reticulosis

βF1 pagetoid reticulosis

βF1 pagetoid reticulosis

Folliculotropism in CD8 epidermotropic TCL

Folliculotropism in CD8 epidermotropic TCL

Epidermotropism in CD8 epidermotropic TCL

Epidermotropism in CD8 epidermotropic TCL

Epidermotropic small lymphoma cells

Epidermotropic small lymphoma cells


Liver involvement by CD8 cytotoxic TCL

Liver involvement by CD8 cytotoxic TCL

CD3 positive lymphoma cells

CD3 positive lymphoma cells

CD8 positive lymphoma cells

CD8 positive lymphoma cells

Molecular / cytogenetics description
Sample pathology report
  • Ulcerated plaque of the trunk, skin punch biopsy:
    • Primary cutaneous T cell lymphoma, most consistent with primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma (see comment)
    • Comment: Section shows an adequate skin punch biopsy with intense epidermotropism, focal epidermal necrosis, acanthosis and spongiosis. There is an extensive, nodular and diffuse dermal infiltrate composed of variably sized, pleomorphic lymphocytes with irregular nuclear contours, hyperchromatic nuclei and scant cytoplasm. Immunohistochemical studies show that the abnormal lymphocytes are diffusely positive for CD3, CD7, CD8, CD45RA, TIA1, T cell receptor beta (βF1) and granzyme B. The atypical cells are negative for CD2, CD4, CD5, CD56, EBER, CD30, T cell receptor gamma / delta and ALK1. The Ki67 proliferation index is approximately 70%. According to clinical notes, the patient is a 63 year old man with history of hypertension. The patient presented to the department of dermatology with a 5 week history of ulcerated plaques, papules and nodules in the upper and lower limbs, trunk, head, neck, tongue and perioral area. A computed tomography (CT) showed multiple nodules in liver, without evidence of lymphadenopathy. The final diagnosis is most consistent with primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma.
Differential diagnosis
Board review style question #1
    What immunophenotype is more commonly expressed by primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma?

  1. CD3+ / CD4- / CD7- / CD8+ / CD30- / EBER-
  2. CD3+ / CD4- / CD7+ / CD8+ / CD30- / EBER+
  3. CD3+ / CD4+ / CD7+ / CD8+ / CD30- / EBER+
  4. CD3+ / CD4+ / CD7+ / CD8+ / CD30+ / EBER-
Board review answer #1
A. CD3+ / CD4- / CD7- / CD8+ / CD30- / EBER-

Comment Here

Reference: Primary cutaneous CD8+ aggressive epidermotropic T cell lymphoma
Board review style question #2
    What is a consistent feature for the diagnostic criteria of primary cutaneous CD8+ aggressive epidermotropic T cell lymphoma?

  1. Pyoderma gangrenosum-like
  2. Spongiosis, blistering and necrosis
  3. Variable expression of CD2, CD4, CD5, CD7 and CD8
  4. Widespread ulcerated papules, plaques and tumors without precursor lesion
Board review answer #2
D. Widespread ulcerated papules, plaques and tumors without precursor lesion

Comment Here

Reference: Primary cutaneous CD8+ aggressive epidermotropic T cell lymphoma
Back to top
Image 01 Image 02