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Year : 2016  |  Volume : 59  |  Issue : 1  |  Page : 131-132
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma: A case report and significance of timely ancillary testing

1 Department of Histopathology, Metropolis Healthcare Limited, Mumbai, Maharashtra, India
2 Department of Molecular Pathology, Metropolis Healthcare Limited, Mumbai, Maharashtra, India

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Date of Web Publication9-Mar-2016

How to cite this article:
Kavishwar VS, Kirti C, Kush R, Sunita K, Pratiksha C. Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma: A case report and significance of timely ancillary testing. Indian J Pathol Microbiol 2016;59:131-2

How to cite this URL:
Kavishwar VS, Kirti C, Kush R, Sunita K, Pratiksha C. Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma: A case report and significance of timely ancillary testing. Indian J Pathol Microbiol [serial online] 2016 [cited 2021 Oct 23];59:131-2. Available from: https://www.ijpmonline.org/text.asp?2016/59/1/131/174881


A 70-year-old man was admitted to the Medical Intensive Care Unit with respiratory tract infection, sepsis, and acute kidney injury; not responding to treatment. Six months back, he had presented with maculopapular skin lesions on the upper and the lower limbs. A skin biopsy had raised the possibility of chronic lymphocytic vasculitis in the primary laboratory. No immunohistochemistry (IHC) was done at that time. As the patient deteriorated, a paraffin block of the same biopsy was submitted to our laboratory for a lymphoma IHC panel 6 months later.

Morphology on routine H and E microscopic examination showed a cutaneous lymphoproliferative lesion displaying dermal nodular aggregates of atypical lymphoid cells surrounding the adnexal structures, and neurovascular bundles along with pagetoid type of epidermotropism [Figure 1]a and b].

On IHC the lesional cells were positive for CD3 [Figure 1]c] with a high Ki-67 index, [Figure 1]d] CD8 predominance [Figure 2]a] and negative for CD30 [Figure 2]b], CD20, and CD4.
Figure 1: (a) Dense dermal lymphoid infiltrate and focal epidermotropism (H and E stain, ×100). (b) Dermal lymphoid aggregates (H and E stain, ×400). (c) Immunohistochemistry - CD3 positive lesional cells (×100). (d) Immunohistochemistry - Ki-67 is high index (×400)

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Figure 2: (a) Multiplex immunohistochemistry CD4+ CD8 cocktail: Atypical T-lymphoid cells show strong red positivity for CD 8 and are CD4 negative (×100). (b) Immunohistochemistry - CD 30 is negative (×400). (c) Polyacrylamide gel for T-cell receptor gamma gene rearrangement. Lane 1: 100 bp ladder; Lane 2: Positive or monoclonal control (167 bp); Lane 3: Specimen control ladder (presence of all the products indicate the integrity of DNA). Lane 4: T-cell receptor gamma MMx-A (shows monoclonal band); Lane 5: T-cell receptor gamma MMx-B; Lane 6: Negative or polyclonal control

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T-cell receptor gamma gene rearrangement confirmed T-cell origin and clonality [Figure 2]c].

Amalgamating clinical presentation, histomorphology, IHC, and molecular assay results, a conclusive diagnosis of primary cutaneous aggressive epidermotropic CD8+ cytotoxic, T-cell lymphoma was established. This lymphoma represents <1% of all cases of cutaneous T-cell lymphoma (CTCL) that has been classified as a nonspecific, provisional entity according to the WHO/EORTC classification in 2005, and the 4 th edition of the WHO classification in 2008. [1],[2] Less than 50 cases have been reported worldwide and probably this is the first Indian patient.

It was first reported by Berti et al., in 1999. He described this tumor as a distinct clinicopathological entity with an aggressive clinical behavior. It is characterized by widespread rapidly evolving papules, plaques, and papulonodules, often showing central necrosis and ulceration. [3] Uncommonly, it may present as multiple maculopapular eruptions on the hands, feet, and face. [4]

Patients are usually adults with a slight male predominance. Our patient was an elderly gentleman who presented with maculopapular skin lesions.

Nofal et al. [5] proposed two sets of diagnostic criteria using a combination of clinical, histopathological, and IHC features.

Constant features - all of constant clinical, histopathological, and IHC features must be present to establish a definite diagnosis.


  1. History : Short; few weeks or months
  2. Course : Aggressive
  3. Lesions : Widespread papules, plaques, and tumors often ulcerated, without any precursor lesions.


  1. Epidermotropism, often prominent
  2. Nodular or diffuse infiltrates of pleomorphic T-cell.


  1. CD8+
  2. CD4–.
Variable features - helps to avoid missing cases.

Clinical (these cases showed classic constant lesions, in addition to uncommon variable features):

  1. Pyoderma gangrenosum: Like lesions
  2. Pagetoid reticulosis: Like superficial hyperkeratotic patches and plaques
  3. Annular erythematous scaling patches.


  1. Spongiosis, blistering, and necrosis
  2. Deep infiltration reaching subcutaneous fat with rimming of adipocytes
  3. Invasion and destruction of adnexal skin structures
  4. Angiocentricity and angioinvasion.

1. Variable expression profiles of CD2, CD5, CD7, CD15, CD30, CD45, and CD56.

Conventional therapies for classic CD4+ CTCL are usually ineffective in this type of lymphoma, which requires multiagent chemotherapy and bone marrow transplantation. Hence, accurate and timely diagnosis is necessary.

The clinical course is usually aggressive with a high tendency for systemic spread to extracutaneous sites such as central nervous system, lung, and testis while lymph nodes are usually spared. The prognosis of the patients is poor with a median survival of 32 months from diagnosis.

As depicted through this case report, it would be logical and wise to conclude that reflex

ancillary tests performed on a primary biopsy provide an early and conclusive diagnosis catalyzing timely optimal patient management.

This patient presented with a cutaneous lymphoma, which was not suspected or confirmed in the initial stage. He had multisystem involvement over a period of 6 months, and, unfortunately, succumbed to the disease on the day he received his final diagnosis.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 1
Asher RG, Hollowood K. Primary cutaneous lymphoma: An overview based on the WHO-EORTC classification. Diagn Histopathol 2010;16:168-81.  Back to cited text no. 2
Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol 1999;155:483-92.  Back to cited text no. 3
Kim SK, Kim YC, Kang HY. Primary cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma with atypical presentation. J Dermatol 2006;33:632-4.  Back to cited text no. 4
Nofal A, Abdel-Mawla MY, Assaf M, Salah E. Primary cutaneous aggressive epidermotropic CD8+T-cell lymphoma : Proposed diagnostic criteria and therapeutic evaluation. J Am Acad Dermatol 2012;67:748-59.  Back to cited text no. 5

Correspondence Address:
Vikas S Kavishwar
Department of Histopathology, Metropolis Healthcare Limited, Central Laboratory, Fourth Floor, Commercial Building 1A, Kohinoor Mall, Vidyavihar (West), Mumbai - 400 070, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.174881

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  [Figure 1], [Figure 2]

This article has been cited by
1 Cutaneous Lymphoma—Inpatient Considerations
Kerry Heitmiller,Jeffrey Barnes,Steven T. Chen
Current Dermatology Reports. 2017; 6(2): 63
[Pubmed] | [DOI]


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