A Rare Case of Epstein-Barr Virus-Positive Peripheral T-Cell Lymphomas (PTCLs) Presenting with Single Large Ulcerative Growth.

Sir,

A 52-year-old male patient presented to dermatology outpatient department with swelling over left inguinal region since 2 months, which was small to start initially and gradually increased over a period of 2 months. One month later swelling ruptured to form a raw area with discharge of clear fluid. This was associated with significant weight loss. Patient also gave history of unprotected sexual contact with commercial sex worker.

Cutaneous examination revealed single, large, non tender, firm swelling measuring about 4 cm × 5 cm × 7 cm with foul smelling yellow base punched out ulcer. There were multiple necrosed areas on swelling which present over his left inguinal region, and multiple well defined hyperpigmented verrucous plaque present over thigh, penis, scrotum, and pubic region [Figure 1]. There was no other skin, oral, or genital lesion. General examination was normal except for inguinal lymphadenopathy. Systemic examination revealed mild splenomegaly. From the above findings a differential diagnosis of lymphoma and lymphogranuloma venereum were considered. Complete blood investigation was within normal limit except for anemia (Hb-8.4 g/dl). Peripheral smear revealed atypical lymphocytes. Rest other hematological investigations were normal. Serology test for human immunodeficiency virus was positive. The Venereal Disease Laboratory Test was nonreactive. Ultrasonography of abdomen was suggestive of mild splenomegaly and left inguinal lymphadenopathy.

Figure 1

Single, large, non tender, firm swelling with yellow base punched out ulcer and multiple necrosed area on swelling present over left inguinal region

Fine needle aspiration cytology from left inguinal swelling revealed moderately cellular smear showing necrotic material in background and polymorphus lymphocytic cell with predominantly large centroblastic cells seen. Many mitotic figures were seen. Skin biopsy from left inguinal swelling showed mild irregular acanthosis with spongiosis. Upper dermis showed dilated blood vessel and perivascular collection of mixed inflammatory infiltrate. Deep dermis showed mononuclear cells in sheets at perivascular location with round to oval nuclei and fine chromatin [Figures 2 and 3]. These findings were suggestive of Non-Hodgkin's lymphoma (NHL). Skin biopsy from hyperpigmented verrucous plaque (thigh) revealed irregular acanthosis, papillomatosis, keratin invaginations, and foci of melanin pigment deposition. These findings were suggestive of seborrheic keratosis. Immunohistochemistry studies showed positive staining for CD4, CD45RO, MUM-1, Ki67, CD30, and EBER-ISH and negative staining for CD3, CD10, CD20, CD138, Bcl-6, CD79a, ALK-1, CD8, and EMA [Figure 4].

Figure 2

Histopathology (×4, H and E) - epidermis shows irregular acanthosis and spongiosis. Dermis shows sheets of monomorphic mononuclear cells

Figure 3

Histopathology (×40, H and E) – mononuclear cells in sheets with round to oval nuclei and fine chromatin present in dermis

Figure 4

IHC staining positive for CD 4, CD 45RO, MUM- 1, Ki67, CD30, and EBER-ISH

Considering the clinical features, histological findings and immunohistochemistry findings a final diagnosis of epstein barr virus (EBV) positive nodal peripheral T-cell lymphoma (PTCL) unspecified type was made. Patient was referred to radiotherapy department for initiation of appropriate chemotherapy.

T-cell lymphomas account for 10%–15% of all NHL.[1] Although PTCL generally affect people older than 60 years, it can occurs in any age groups.[1] Epstein–Barr virus associated PTCL is more common in East Asia than in Western populations.[2]

PTCL appear to be quite heterogenous in clinical manifestation, pathological findings, and outcomes. Primary cutaneous PTCL, unspecified- refers to a group of diseases that do not fit into any of the other subtypes of PTCL, which present with solitary, localized, or generalized nodules or tumors without predilection for specific locations. Tumor cells are usually CD4-positive.

Although the exact mechanism of action through which EBV causes cancer is unknown, the EBV oncogene, latent membrane protein, is essential for EBV transformation of lymphocytes, which interacts with signal transducer proteins belonging to the tumor necrosis factor (TNF) family of receptors (TRAFs) and inducing nuclear factor NF-κB activation.[3]

EBV-infected T-cells tend to survive and proliferate by upregulating the T-cell-activating receptor CD40 and major histocompatibility complex class II, while down regulating the cell death surface receptor first apoptosis signal (FAS).[3]

There is paucity of literature describing this entity [Table 1]. In this study, we report a case of EBV positive PTCL because of its rarity as it present with single inguinal lesion over inguinal region in elderly male.

Table 1

Few report of PTCL- unspecified type

Case	Age/sex	Clinical features	Immunohistochemistry
BJ friedman et al.[4]	39 year/M	Multiple ulcerated large nodule on the scalp, forehead, and right side of the face	CD4 positive
V gupta et al.[5]	30 year/M	Multiple tender cutaneous nodule and plaque on the head, trunk, and extremities	CD3 positive
AH pai et al.[6]	26 year/M	Erythematous plaque with large white scale and loss of hair seen abdomen	CD3, CD2, CD5, and CD4 positive
		Erythematous plaque with multiple area of focal atrophy and scaling seen on the left upper arm
Our case	52 year/M	Single, large, non tender, firm swelling with punched out ulcer over left inguinal region	CD4, CD45RO, MUM-1, Ki67, CD30, and EBER-ISH positive

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Conflicts of interest

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