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Table 2 Summary of Reported Cases of EBVMCU disease

From: Diagnostic and therapeutic challenges of EBV-positive mucocutaneous ulcer: a case report and systematic review of the literature

Age Sex Ulcer location Predisposing factor Treatment Response (durability) Ref
Iatrogenic immunosuppression-associated EBVMCU
53 F Colon, rectum Methotrexate + infliximab (CD) Reduced IS PD→HL [75]
56 F Skin (leg) Methotrexate (PM) Reduced IS CR (33 m) [76]
59 F Eyelid Methotrexate (RA) Reduced IS CR (37 mo) [76]
60 F Lip mucosa Methotrexate (RA) Reduced IS CR (72 mo) [20]b
61 F Skin (leg) Methotrexate (RA) R-CHOP CR (25 mo) [76]
62 F Lip, nose, eyelid Methotrexate (PM) Reduced IS CR (1 mo) [45]
64 F Buccal mucosa Methotrexate (RA) Reduced IS Dieda [76]
65 M Palate Methotrexate (RA) Reduced IS CR (19 mo) [77]
69 F Colon Methotrexate (RA) NR NR [20]b
76 F Eyelid Methotrexate (RA) Reduced IS CR (24 mo) [76]
80 M Tongue base Methotrexate (RA) NR NR [20]b
80 F Skin (arm) Methotrexate (RA) Reduced IS CR (60 mo) [20]b
81 F Tongue Methotrexate (RA) Reduced IS CR (12 mo) [78]
42 M Oral mucosa Azathioprine (sarcoidosis + MG) NR NR [20]b
63 M Skin (perianal) Azathioprine (CD) Reduced IS CR (6 weeks) [79]
75 F Esophagus Azathioprine (RA) Reduced IS CR (17 mo) [20]b
76 F Buccal mucosa Azathioprine (Pemphigoid) Reduced IS CR (13 mo) [80]
81 F Colon Azathioprine (ITP) None PD on diagnosis [81]
48 F Tongue Cyclosporine-A (SLE) Reduced IS CR (24 mo) [20]b
64 F Colon Cyclosporine-A (HSCT) Reduced IS CR (6 mo) [20]b
70 M Lip Cyclosporine-A, prednisone (renal transplant) Reduced IS, surgical excision CR (111 mo) [82]
80 F Rectum Cyclosporine-A (UC) Reduced IS CR (23 mo) [20]b
63 F Gingiva Cyclosporine-A, prednisone, MMF (renal transplant) Reduced IS CR (8 mo) [82]
44 M Tongue MMF, pred (renal transplant) Reduced IS CR (15 mo) [82]
45 M Gingiva MMF (SLE) NR NR [83]
61 M Esophagus MMF, pred (renal transplant) Reduced IS CR (16 mo) [82]
70 M Rectum MMF, pred (renal transplant) Reduced IS, rituximab (2 doses) velcade CR (17 mo) [82]
18 M Tonsil, buccal mucosa MMF, prednisone, tacrolimus (cardiac transplant) Reduced IS, rituximab (2 doses) CR (14 mo) [82]
32 M Terminal ileum MMF, prednisone, tacrolimus (bilateral lung transplant) Reduced IS, rituximab (4 doses) CR (60 mo) [82]
Primary Immunodeficiency-Associated EBVMCU
45 F Gingiva T-cell deficiency NOS rituximab (8 doses) CR (24 mo) [62]
61 F Esophagus (multifocal) hypogammaglobulinemia rituximab (4 doses), IVIG (monthly), brentuximab (3 cycles) PD [63]
EBVMCU of Unclear Etiology
49 F Gingiva, palate etiology not established rituximab (4 doses × 2) RT PD CR (6 mo) This case
EBVMCU secondary to age-associated immunosenescence
63 F Tonsil Age NR NR [20]
64 F Tongue, oral mucosa Age RT CR (15 mo) [20]b
68 F Tongue Age None SR (36 mo) [20]b
68 F Tonsil Age R-CHOP, RT CR (24 mo) [20]b
73 M Tonsil, tongue Age None RR (12 mo) [20]b
74 M Skin (neck) Age R-CHOP CR (24 mo) [84]
75 F Skin (arm) Age NR NR [20]b
76 M Tongue base Age None SR (12 mo) [20]b
79 M Skin (cheek) Age None SR (25 mo) [20]b
80 F Palate Age RT CR (60 mo) [20]b
81 F Palate Age None SR (14 mo) [85]
82 F Lip Age None RR (NR) [20]b
82 M Lip Age None SR (NR) [20]b
84 F Tongue, floor of mouth Age None SD (5 mo) [20]b
85 F Tonsil Age RT CR (3 mo) [20]b
88 F Tongue base Age None RR (24 mo) [20]b
88 M Skin (chest) Age None SR (3 mo) [20]b
89 M Tongue base Age NR NR [20]b
89 M Lip, skin (scalp) Age resection NR [86]
101 M Tonsil Age R-CHOP CR (12 mo) [20]b
  1. F female, M male, Ref reference, CD Crohn’s disease, RA rheumatoid arthritis, PM polymyositis, MG myasthenia gravis, ITP immune thrombocytopenia, SLE systemic lupus erythematosus, HSCT hematopoietic stem cell transplant, SOT solid organ transplant, UC ulcerative colitis, HL Hodgkin lymphoma, NOS not otherwise stated, Pred prednisone, MMF mycophenolate, IS immunosuppression, R-CHOP rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, RT radiotherapy, IVIG intravenous immunoglobulin, PD persistent disease, CR complete response, NR not reported, SR spontaneous regression, RR relapsing remitting, Mo months
  2. aDied soon after diagnosis from myelitis and sepsis
  3. bCases are also included in a second published series of EBV-associated lymphoproliferative disorders [87]