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E-IJD® - CORRESPONDENCE
Year : 2021  |  Volume : 66  |  Issue : 2  |  Page : 224
Peripheral T-cell lymphoma-not otherwise specified presenting as disfiguring facial swelling


1 Department of Pathology, Burdwan Medical College, Burdwan, West Bengal, India
2 Department of Dermatology, Burdwan Medical College, Burdwan, West Bengal, India

Date of Web Publication16-Apr-2021

Correspondence Address:
Shreosee Roy
Department of Pathology, Burdwan Medical College, Burdwan, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_205_18

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How to cite this article:
Bandyopadhyay A, Roy S, Seth J, Boler AK, Mitra S, Mishra P. Peripheral T-cell lymphoma-not otherwise specified presenting as disfiguring facial swelling. Indian J Dermatol 2021;66:224

How to cite this URL:
Bandyopadhyay A, Roy S, Seth J, Boler AK, Mitra S, Mishra P. Peripheral T-cell lymphoma-not otherwise specified presenting as disfiguring facial swelling. Indian J Dermatol [serial online] 2021 [cited 2021 May 6];66:224. Available from: https://www.e-ijd.org/text.asp?2021/66/2/224/313780




Sir,

The sudden development of facial swelling and nodules may raise a clinical suspicion of inflammatory or infectious diseases. It can, however, be the first manifestation of an underlying neoplastic process. Here, we describe a patient suspected to have facial cellulitis but later diagnosed as an unusual presentation of peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS).

A 40-year-old diabetic male presented to the dermatology clinic with generalized facial swelling following a facial massage at a salon for 1 month. Physical examination revealed erythema, edema, and tenderness all over the face [Figure 1]a. It was suspected to be a case of facial cellulitis with allergic contact dermatitis and treated with antibiotics with short course of steroid for 5 days as well as antifungals. Steroid was administered to combat respiratory distress and chocking sensation that the patient initially had and also to address the allergic component we suspected. Antifungals were given for coexistent dermatophytosis. After initial improvement, the patient took discharge on risk bond. However, after 2 weeks, he came back again with severe facial edema and larger nodular lesions. Again the patient was put on parenteral antibiotics, but without much response. When the symptoms persisted, subsequent magnetic resonance imaging revealed extreme facial edema and small cervical lymphadenopathy. Fine-needle aspiration cytology of the forehead nodule showed large lymphoid cells in the background of histiocytes, macrophages, and eosinophils. The aspirate was negative for bacterial and fungal cultures and also tuberculosis polymerase chain reaction. Subsequent biopsy of the forehead nodule showed diffuse dermal infiltrates of large lymphoid cells with pleomorphic irregular nuclei and prominent nucleoli. Between the neoplastic cells, there were scattered eosinophils, histiocytes, and few mitotic figures. The neoplastic lymphoid cells infiltrated the subcutis and facial muscle [Figure 1b]. No epidermotropism was seen. On immunohistochemistry, the tumor cells were diffusely positive for CD3, CD4, and BCL2. CD7, CD30, CD25, and CD56 were negative. CD20 and CD8 stained background B and some small T-cells. The case was diagnosed as PTCL-NOS secondarily involving the facial skin. No other lymph node was enlarged, and the bone marrow biopsy was unremarkable. The patient was planned for chemotherapy with CHOP regimen (cyclophosphamide, adriamycin, vincristine, and prednisolone). Unfortunately, the patient died after receiving 1 cycle of chemotherapy due to sudden myocardial infarction.
Figure 1: (a) Clinical photograph of the patient. (b) Histopathological examination of the forehead nodule showed diffuse dermal infiltrates of large lymphoid cells with pleomorphic irregular nuclei and prominent nucleoli. Between the neoplastic cells, there were scattered eosinophils, histiocytes, and few mitotic figures. The neoplastic lymphoid cells infiltrated the subcutis and facial muscle (H and E, ×40)

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PTCL-NOS is a heterogeneous category of nodal and extranodal mature T-cell lymphomas that usually have an aggressive course and respond poorly to therapy.[1] Most commonly, the peripheral lymph nodes are involved. Although extranodal dermal presentation can occur, facial erythema and edema as the initial presentation is very rare.[2] The pathogenesis of this facial swelling in lymphomas is still a topic of debate. The various hypotheses include obstruction of lymphatic ducts, the angiocentric and panniculitis-like growth pattern, and secretion of cytokines by tumor cells.[2],[3]

This unusual presentation as facial cellulitis posed a diagnostic dilemma, and initially an infective etiology was suspected. When the patient did not respond to antibiotics and antifungals, fine-needle aspiration was done. However, cytologic diagnosis is often equivocal as in our case, and hence a surgical biopsy along with immunohistochemistry is essential for definitive diagnosis.[1]

The other lymphomas having facial presentation such as mycosis fungoides and extranodal natural killer/T-cell lymphoma that comprised the differential diagnosis could be excluded since they commonly present with epidermotropic infiltrates by atypical lymphoid cells which was absent in our case.

Thus to conclude, the presence of refractory facial edema may be a preceding event of lymphomas. A high level of clinical suspicion along with serial biopsies will prevent a delay in the diagnosis of rare but aggressive lymphomas such as PTCL-NOS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

The authors are thankful to Dr. Goutam Bandyopadhyay for his help and support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Yao JL, Cangiarella JF, Cohen JM, Chhieng DC. Fine-needle aspiration biopsy of peripheral T-cell lymphomas. A cytologic and immunophenotypic study of 33 cases. Cancer 2001;93:151-9.  Back to cited text no. 1
    
2.
Lee WJ, Moon IJ, Won CH, Chang SE, Choi JH, Moon KC, et al. Facial swelling: An atypical presentation of cutaneous lymphoma. Int J Dermatol 2016;55:e440-6.  Back to cited text no. 2
    
3.
Park GH, Won CH, Chang SE, Lee MW, Choi JH, Moon KC, et al. Extranodal natural killer/T-cell lymphoma preceded by persistent hemifacial swelling. Int J Dermatol 2016;55:450-2.  Back to cited text no. 3
    


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