EBV Positive Mucocutaneous Ulcer—A Study of 26 Cases Associated With Various Sources of Immunosuppression

Abstract

We describe a series of Epstein Barr virus (EBV)-positive circumscribed, ulcerative lesions associated with various types of immunosuppression (IS). The study group (26 patients) comprised 10 males and 16 females, median age 77 years (range 42 to 101). IS in 9 cases included azathioprine (AZA), methotrexate (MTX) or cyclosporin-A (CyA). Seventeen patients had age-related immunosenescence. Patients presented with isolated sharply circumscribed ulcers involving oropharyngeal mucosa (16), skin (6), and gastrointestinal tract (4). Lesions were histologically characterized by a polymorphous infiltrate and atypical large B-cell blasts often with Hodgkin/Reed-Sternberg (HRS) cell-like morphology. The B cells showed strong CD30 and EBER positivity, some with reduced CD20 expression, in a background of abundant T cells. CD15 was positive in 43% of cases (10/23). The pathologic features were identical regardless of the anatomic site or cause of IS. Polymerase chain reaction revealed 39% (7/18) clonal Ig gene rearrangements with 38% (6/16) and 31% (5/16) clonal and restricted T-cell patterns, respectively. Twenty-five percent of patients (5/20) received standard chemotherapy and/or radiotherapy. Forty-five percent (9/20) regressed spontaneously with no treatment and 15% (3/20) were characterized by a relapsing and remitting course. All of the iatrogenic lesions (6/6) with available follow-up responded to reduction of IS. All patients achieved complete remission with no disease-associated deaths over a median follow-up period of 22 months (range 3 to 72). We propose EBV-positive mucocutaneous ulcer as a newly recognized clinicopathologic entity with Hodgkin-like features and a self-limited, indolent course, generally responding well to conservative management. Association with various forms of IS implies a common pathogenetic mechanism. The localized nature of the disease may be owing to a minimal and localized lapse in immunosurveillance over EBV.

Viral loads and antiviral resistance of herpesviruses and oral ulcerations in hematopoietic stem cell transplant recipients

“…Recently, EBV-positive oral ulcers with Hodgkin's-like features that responded well to reduction of immunosuppression have been described in immunosuppressed patients, including one HSCT recipient. 34 Also, EBV-associated oral lesions resembling oral hairy leukoplakia with extensive mucosal ulceration have been described in an HSCT recipient. 16 In our patient group, oral EBV shedding was not a predictor of systemic EBV complications, as there was no difference in the occurrence of EBV plasma DNAemia or the occurrence of post-transplant lymphoproliferative disorder between patients with or without EBV in the oral samples (data not shown).…”

New developments in the pathology of malignant lymphoma: a review of the literature published from October 2009 to January 2010

“…Another EBV-associated lesion is described by Dojcinov et al [52]. A series of 26 patients with immunosuppression had an EBV-positive circumscribed, ulcerative lesion.…”

Primäres Hodgkin-Lymphom des Kolons

“…Im hier vorliegenden sehr seltenen Fall eines primär intestinalen EBV-assoziierten Hodgkin-Lymphoms bei einer älteren Patientin sind 2 Differenzialdiagnosen näher zu diskutieren: F Abgrenzung gegenüber einer so genannten "EBV-assoziierten mukosalen Ulzeration" [3], F Abgrenzung gegenüber einem "EBV-positiven diffus großzelligen Lymphoms des älteren Patienten" [8].…”

“…Darmerkrankungen (CED), welche häu-fig unter immunsuppressiver Medikation stehen, eigentlich dieser erst kürzlich beschriebenen Entität zuzuordnen wären [3,7]. Bei der hier beschriebenen Patientin war keine Immunsuppression im en- …”