Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma

Indian J Ophthalmol. 

doi:  10.4103/0301-4738.151496

PMCID: PMC4363975

Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma on upper eyelid

Joon Lee, You Jin Yang, Jung-In Kim, Ji-Hye Park, and Won-Serk Kim

Department of Dermatology, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea

Address for correspondence: Won-Serk Kim, 108, Pyeong-dong, Jongno-gu, Seoul -110-746, Korea. E-mail: moc.revan@inisus

Sir,

Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma constitutes less than 1% of all cases of cutaneous T-cell lymphoma (CTCL).[1] Although common subtypes of CTCL, such as mycosis fungoides (MF), are originated from CD4 + memory T cells, this subtype is derived from a cytotoxic T-cell subset and follows a more aggressive clinical course with poor prognoses.[2]

An 80-year-old man was referred with the well-demarcated, indurated plaque with central necrotic ulceration on his left upper eyelid [Fig. 1]. He developed a single, erythematous lesion with on left upper eyelid 2 years ago. In a tertiory hospital, he was diagnosed with pseudolymphoma and had been treated with steroid intralesional injection and tacrolimus ointment. During the next year, his disease progressed and the lesion tended to bleed and he had to use an eye patch. On exam, no afferent pupillary defect, diplopia, or proptosis was present. Slit lamp examination was unremarkable. He could not elevate the upper eyelid due to mass. There was no involvement in the conjunctiva, upper fornix, and ocular surface.

Figure 1

A slightly indurated well-demarcated ulcer, slightly pearly in periphery

A biopsy was performed and histology showed appearances suggestive of a malignant lymphoma [Fig. 2]. Skin biopsy specimen revealed a bandlike, epidermotropic, and perivascular atypical lymphocytic infiltration with large-cell morphology, which extends to the reticular dermis and subcutis. The tumor cells were positive for CD3, CD8, Bcl-2, CD56, and granzyme B (in a few scattered cells only), and negative for CD10, CD25, CD20, CD30. In situ hybridization for Epstein-Barr virus was negative. HIV testing and the test for monoclonal rearrangement of the T-cell antigen receptor genes were not performed. Other organ involvement was not found at a computed tomography and PET-CT. He was diagnosed with primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma and transferred to the oncologist for the combined chemotherapy with CHOP (cyclophosphamide, adriamycin, vincristine, prednisone). However, the treatment was not effective after three cycles of the combined chemotherapy. We planned to perform the radiotherapy and flap surgery, but the patient refused the surgery because of his general medical conditions such as previous myocardial infarction and diabetes.

Figure 2

(a) Punch biopsy of upper eyelid lesion showed epidermal ulceration, with underlying pandermal and epidermotropic lymphocytic infiltrate, composed nearly entirely of large atypical lymphocytes, extending to and involving subcutis (H&E, ×40). ...

There are approximately 30 cases of on primary cutaneous aggressive epidermotropic CD8 + cytotoxic T-cell lymphoma reported in the literature, and most of the cases represented multiple or widespread papules, nodules, and tumors, often with hemorrhage, ulceration, and necrosis.[3] One case of solitary ulcerated lesion at ear pinna similar to this case was reported by Fika et al.[4] The prognosis is very poor, according to studies by Berti et al.,[2] which found median survival to be only 32 months. This case generally follows an indolent clinical course similar to that of the more common, classic CD4 + variants. The other case, reported by Fika et al.,[4] showed solitary lesion, with slow progression, excellent responsiveness to local radiotherapy. The CD8 + immunophenotypic variant of MF, reported by Dummer et al.,[5] is also characterized by a slowly progressing course, responsiveness to conservative therapy. The significant histologic and immunophenotypic overlap between the more indolent and aggressive cases suggest that differential diagnosis must be made based on clinical features and disease course. These indolent variants must be discriminated from the more aggressive CD8 + lymphoma to prevent aggressive treatment.

This case report is additional information to the data collected on primary cutaneous aggressive epidermotropic CD8 + cytotoxic T-cell lymphoma. In this case, the clinical course did not show an aggressive progression and solitary lesion on upper eyelid without extracutaneous manifestation unlike the most of the cases reported as primary cutaneous aggressive epidermotropic CD8 + cytotoxic T-cell lymphoma, which have no effective treatment at present.

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References

1. 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. [PubMed]

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. [PMC free article] [PubMed]

3. Gormley RH, Hess SD, Anand D, Junkins-Hopkins J, Rook AH, Kim EJ. Primary cutaneous aggressive epidermotropic CD8+T-cell lymphoma. J Am Acad Dermatol. 2010;62:300–7. [PubMed]

4. Fika Z, Karkos PD, Badran K, Williams RE. Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma of the ear. J Laryngol Otol. 2007;121:503–5. [PubMed]

5. Dummer R, Kamarashev J, Kempf W, Häffner AC, Hess-Schmid M, Burg G. Junctional CD8+cutaneous lymphomas with nonaggressive clinical behavior: A CD8+variant of mycosis fungoides? Arch Dermatol. 2002;138:199–203. [PubMed]

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