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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]