Primary cutaneous extranodal NK/T-cell lymphoma, nasal type, in an adolescent

ARTICLE

Auteur(s) : Ping Wang¹, Yan Sun¹, Zhaoming Wang², Aie Xu¹

¹Department of Dermatology, the Affiliated 3rd Hospital of Hangzhou, Anhui Medical University, Hangzhou, 310009, China
²Department of Pathology, the 1st Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, 310009, China

A 15-year-old girl was admitted to our hospital in April 2002, with a nearly two year history of repeated intense skin reactions to insect and mosquito-bites. Each rash began with asymptomatic edematous erythemas on the face and lower extremities, some lesions became bullae and sometimes evolved into painful ulcers. The lesions self-cured after 4-5 days with pigmentation and scars remaining. No fever, malaise, or other systemic symptoms were noticed. At the time of the first admission, physical examination showed marked swelling on the right face and upper lip, multiple edematous erythemas, pigmentation and scars scattered over the face, trunck and extremities. A central-crusted nodule and centrifuged enlarged edematous erythemas with tiny scaling on the upper extremities were also noticed (figures 1A, B). Enlarged lymph nodes were not present on palpation. Routine laboratory and naso-endoscopic examination and head CT scaning were normal. Abdominal ultrasonic B showed splenomegaly. A skin biopsy from the edematous erythema demonstrated perivascular moderate infiltrates composed of neutrophils, eosinophils and lymphoid cells in the dermis consistent with arthropod bite reactions. The patient was diagnosed as hypersensitivity to mosquito bites and treated with oral prednisolone and glycyrrhizin with some benefit. One and half years later, the patient was hospitalized again for worsened rashes, high fever, fatigue and weight loss. Physical examination showed multiple bullae and ulcers with irregular margins and different depths on her trunks and extremities (figure 1C). Serum antibodies for virus infection were positive for EBV IgG 1:320. Biopsy specimens taken from the edge of an ulcer on the leg and an erythema plaque on the back respectively revealed similar features, with sub-epidermal blisters and moderate angiocentric infiltrates of atypical lymphoid cells (figures 1D, E). These cells were positive for CD3, CD56, CD45RO, EBNA (figure 1F), weakly positive for TIA, and negative for CD20, CD4, CD8, CD30, CD34, and CD68. A polymerase chain reaction showed germline configuration of TCRγ gene rearrangement in the cutaneous lesion. The patient was diagnosed with extranodal NK⁄T-cell lymphoma, nasal type. She was then sent to Tumor Hospital of Zhejiang Province, treated with cyclophosphamide, vincristine, and prednisone together with interferon-a 2b, but showed no improvement. One month later, she died.

Extranodal NK/T-cell lymphoma, nasal type (ENKL) shows a mark geographical preference for East Asia, including China, and Latin America [1]. It is well recognized as a distinct lymphoma with characteristic clinical features, histopathology, immunophenotype and relation with Epstein-Barr virus (EBV) infection. ENKL shows a predilection for the upper aerodigestive tract, representing more than 80% of this tumor. The skin is the second most frequent organ of origin, accounting for approximately 10% of cases [2].

Clinical presentation of primary cutaneous ENKL is non-specific, usually presenting as a single or multiple nodules or tumors that persist and progress over time. Rarely, as in our case, the clinical onset was severe mosquito bite reactions with ulcer-necrotic lesions, later presenting with centrifuged enlarged edematous erythemas. At the advanced stage, the multiple bullae formation and ulceration resembled pyoderma gangrenosum. Other rare specific features, such as ENKL mimicking pyogenic granuloma [3], or cellulites [4] can also be seen.

ENKL is associated with an aggressive clinical course with a median survival of approximately one year after diagnosis, despite chemotherapy and radiation therapy. However, Nitta et al. [5] reported a 14-year-old Japanese girl with EBV-associated NK/T cell lymphoproliferative disorders with a 12-year clinical course. Our case also had a 5 year history. This may be due to a prolonged latent stage EBV infection with no systemic involvement, especially in children, before the advanced tumor stage emerges.

Acknowledgments

References

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3 Fernández-Torres R, Del Pozo J, Alvarez A, et al. Extranodal NK/T-cell lymphoma, nasal type presenting as a pyogenic granuloma-like on a fingertip. Eur J Dermatol 2009; 19: 79-80.

4 Jia H, Sun T. Extranodal NK/T-cell lymphoma mimicking cellulitis. Leuk Lymphoma 2004; 45: 1467-70.

5 Nitta Y, Iwatsuki K, Kimura H, et al. Fatal natural killer cell lymphoma arising in a patient with a crop of Epstein-Barr virus-associated disorders. Eur J Dermatol 2005; 15: 503-6.