Indian Journal of Dermatology
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CASE REPORT
Year : 2010  |  Volume : 55  |  Issue : 3  |  Page : 279-280
Cutaneous involvement in angioimmunoblastic T-cell lymphoma


Department of Dermatology, Internal Medicine & Pathology, University of Athens, ATIIKON General Hospital, Athens, Greece

Date of Web Publication25-Sep-2010

Correspondence Address:
Evangelia Papadavid
Dermatology Department, University of Athens, ATIIKON General Hospital, Athens, 12462
Greece
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.70704

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   Abstract 

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin's nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia, and cutaneous involvement. We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression.


Keywords: Angioimmunoblastic T-cell lymphoma, cutaneous involvement


How to cite this article:
Papadavid E, Panayiotides I, Dalamaga M, Katoulis A, Economopoulos T, Stavrianeas N. Cutaneous involvement in angioimmunoblastic T-cell lymphoma. Indian J Dermatol 2010;55:279-80

How to cite this URL:
Papadavid E, Panayiotides I, Dalamaga M, Katoulis A, Economopoulos T, Stavrianeas N. Cutaneous involvement in angioimmunoblastic T-cell lymphoma. Indian J Dermatol [serial online] 2010 [cited 2020 Feb 29];55:279-80. Available from: http://www.e-ijd.org/text.asp?2010/55/3/279/70704



   Introduction Top


The cutaneous findings of angioimmunoblastic T-cell lymphoma (AITL) most commonly consist of a maculopapular eruption on the trunk mimicking toxic erythema. The rash is persistent and usually misdiagnosed as toxic erythema due to drug eruption or viral exanthem. [1] A skin biopsy reveals histological and immunohistochemical findings and leads to the diagnosis of cutaneous AITL. In doubtful cases, molecular studies of clonal TCR-g-chain gene rearrangement in the skin and lymph nodes are helpful. [2],[3]


   Case Report Top


A 61-year-old man with a 2-year history of AITL presented to the Dermatology Department with a widespread macular erythema on his trunk and extremities mimicking toxic erythema [Figure 1]. The rash, first appeared at the time of diagnosis, was persistent and misdiagnosed as toxic erythema due to drug eruption and disappeared only with complete remission after chemotherapy COP. After two years, the rash relapsed with AITL progression. His virology tests for recent EBV, CMV, HSV1, HSV2, HZV, HTLV-1, and HTLV-2 infections were negative. A skin biopsy performed at disease relapse was diagnostic of skin involvement in AITL. The epidermis did not show any specific changes, but a dense perivascular cellular infiltration consisting of mature lymphocytes, histiocytes, a small number of eosinophilic polymorphonuclear leucocytes, and few small to medium size lymphoid cells with oval or cleaved nucleus was seen in the dermis. The majority of the cells were positive for CD3 [Figure 2] and negative for CD79a, CD68/PGM1, CD30, CD4, and CD8. The patient's condition progressed despite chemotherapy and had a fatal course few months after the second relapse.
Figure 1: Widespread macular erythema on the trunk

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Figure 2: A dense perivascular cellular infiltration consisting of mature lymphocytes, histiocytes, a small number of eosinophilic polymorphonuclear leucocytes and few small to medium size lymphoid cells with oval or cleaved nucleus positive for CD3 was seen in the dermis (CD3, × 20)

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   Discussion Top


AITL is an aggressive non-Hodgkins nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia and cutaneous involvement. The cutaneous findings most commonly consist of a maculopapular eruption on the trunk. However, purpura, infiltrated or urticarial plaques, papulovesicular lesions, nodules, and erythroderma have also been reported. [1] Forty-four percent of patients with AITL experience a nonspecific maculopapular dermatitis, which precedes other clinical symptoms by at least several weeks [1] suggesting that AITL should be included in the differential diagnosis of any maculopapular eruption of unknown etiology accompanied by lymphadenopathy. Histologic findings in the lymph node are characteristic, while those in the skin may be very subtle, showing only mild lymphoid infiltrate. In our case, the clinical history of AITL in addition to the clinical, histological, and immunohistochemical findings led to the diagnosis of cutaneous AITL. In doubtful cases, molecular studies of clonal TCR-g-chain gene rearrangement in the skin and lymph nodes are helpful. [2],[3] Although its pathogenesis is not clear, AITL is a peripheral T-cell lymphoma of mature T-cells secreting several cytokines, which may help in predicting the biological behaviour of such an aggressive disease. [4]

We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression. Given the allergy of the patient to the most frequently administered antibiotics, the risk of misdiagnosing the origin of such an eruption is present. In most of the cases, awareness of the history of AITL with ancillary studies, including clonality testing for T-cell receptor gene rearrangement, is crucial for reaching an accurate diagnosis. [3],[4]

 
   References Top

1.Bernstein JE, Soltani K, Lorincz AL. Cutaneous manifestations of angioimmunoblastic lymphadenopathy. J Am Acad Dermatol 1979;1:227-32.  Back to cited text no. 1  [PUBMED]    
2.Yoon GC, Chang SE, Kim HH, Choi JH, Sung KJ, Moon KC. Kim Cutaneous relapse of angioimmunoblastic lymphadenopathy-type peripheral T cell lymphoma mimicking an exanthematous drug eruption. Int J Dermatol 2003;42:816-8.  Back to cited text no. 2      
3.Huang CT, Chuang SS. Angioimmunoblastic T-cell lymphoma with cutaneous involvement: a case report with subtle histologic changes and clonal T-cell proliferation. Arch Pathol Lab Med 2004;128:e122-4.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Murakami T, Ohtsuki M, Nakagawa H. Angioimmunoblastic lymphadenopathy-type peripheral T cell lymphoma with cutaneous infiltration: report of a case and its gene expression. Br J Dermatol 2001;144:878-84.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2]

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures

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