Table 2.
Clinical features and histopathologic findings in patients with hydroa vacciniforme-like lymphoproliferative disease at our institution.
| Patient no. | Sex | Age at diagnosis, years (age at onset, years) | Cutaneous manifestations | IHC, EBER-1 ISH, TCRγ gene rearrangement | Serum EBV DNA load (copies/mL) | UVA provocation test | Extracutaneous involvement | Treatment | Disease course | Follow-up (months) | Last status during follow-up |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 7 (6) | Erythematous crusted papulovesicles on the face and oral ulcer | CD3+, CD20+ a few, Ki-67 10%, EBV ISH+ | 15,462,763 | Positive | None | Observation | Partial remission and recurrence | 72 | AWD |
| 2 | M | 10 (9) | Erythematous crusted papulovesicles on the face | CD3+, CD20+ a few, CD56−, EBV ISH+ | ND | Negative | None | Systemic steroid, minocycline | Partial remission and recurrence | 216 | AWD |
| 37 | F | 11 (5) | Erythematous crusted papulovesicles on the face and arm, and oral ulcer | CD3+, CD45RO+, CD8+, CD20−, CD56+ few, LMP-1−, EBV ISH+ | 1,784,901 | ND | None | Systemic steroid on recurrence | Partial remission and recurrence | 180 | AWD |
| 48 | F | 12 (3) | Erythematous crusted papulovesicles on the face, arm, and leg | CD3+, CD45RO+, CD56−, S-100−, lysozyme+ focal, EBV ISH+ | ND | ND | Liver, spleen, bone marrow | Oral acyclovir | Progressed to aggressive NK-cell leukemia | 108 | DOD |
| 59 | F | 15 (10) | Erythematous crusted papulovesicles on the face and arm | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67+ a few, LMP-1−, EBV ISH+, TCR gamma gene arrangement− | ND | Negative | None | Observation | Remission | 36 | AWD |
| 6 | M | 17 (17) | Erythematous crusted papulovesicles on the face and trunk, and oral ulcer | CD3+, CD4+, CD8+ focal, CD20−, CD56−, TIA-1+, Ki-67 70%, EBV ISH+, TCR gamma gene rearrangement− | 8,463,700 | ND | Bone marrow | Systemic steroid → chemotherapy | Progressed to systemic EBV+ T-cell lymphoma | 12 | AWD |
| 710 | M | 17 (8) | Erythematous crusted papulovesicles on the face and forearm | CD45RO+, CD20−, EBV ISH+ | ND | Negative | Cervical lymph node, liver, spleen | Systemic steroid, chemotherapy | Progressed to systemic EBV+ T-cell lymphoma | NS | AWD |
| 8 | M | 18 (6) | Erythematous crusted papulovesicles on the face, arm, and trunk | CD3+, CD4+, CD8+ focal, CD20−, CD30+ a few, CD56+, granzyme B+, Ki-67 10%, EBV ISH+ | 47,925,563 | Positive | Cervical lymph node, spleen, iliac bone, bone marrow | Systemic steroid → chemotherapy | Progressed to systemic EBV+ T-cell lymphoma | 12 | DOD |
| 9 | F | 19 (17) | Erythematous crusted papulovesicles on the face and arm, oral ulcer, and facial swelling | CD3+, CD4−, CD8−, CD20−, CD56−, CD30−, EBV ISH+ | 693,642 | ND | right breast, lung, spleen, liver, pelvic bone, bone marrow | Systemic steroid → chemotherapy | Progressed to extranodal NK/T-cell lymphoma, nasal type | 24 | DOD |
| 10 | M | 19 (4) | Erythematous crusted papulovesicles on the face, arm, trunk, and ankle | CD20+ a few, CD56+ a few, LMP-1−, EBV ISH+ | 79,396 | ND | None | Observation | Remission | 204 | AWD |
| 1110 | M | 19 (4) | Erythematous crusted papulovesicles on the face and forearm | CD45RO+, CD20−, EBV ISH+ | ND | Negative | None | Systemic steroid, oral acyclovir | Partial remission | NS | AWD |
| 1210 | M | 21 (19) | Erythematous crusted papulovesicles on the face and oral ulcer | CD45RO+, CD20−, EBV ISH+ | ND | Negative | Cervical lymph node, liver | Systemic steroid | Progressed to Systemic EBV+ T-cell lymphoma | NS | DOD |
| 13 | M | 24 (10) | Erythematous crusted papulovesicles on the face, arm, and trunk | CD3+, CD45RO+, CD4+, CD8+, CD20+ a few, CD56−, granzyme B+ a few, Ki-67 1%, EBV ISH+, TCR gamma gene rearrangement− | 1,840,744 | ND | Liver, spleen, bone marrow | Systemic steroid → chemotherapy | Progressed to Systemic EBV+ T-cell lymphoma | 120 | DOD |
| 1410 | M | 24 (18) | Erythematous crusted papulovesicles on the face and forearm | CD45RO+, CD20−, EBV ISH+ | ND | Negative | Cervical lymph node, spleen | Systemic steroid | Progressed to peripheral T-cell lymphoma of adulthood | NS | DOD |
| 15 | M | 26 (25) | Erythematous crusted papulovesicles on the face | CD3+, CD4+, CD8+ focal, CD 20+ a few, CD30+ focal, CD56−, Ki-67 15%, TCR gamma gene rearrangement+ | 30,917,751 | ND | Cervical and axillary lymph node, spleen | Systemic steroid → chemotherapy | Progressed to peripheral T-cell lymphoma of adulthood | 204 | AWD |
| 169 | M | 28 (22) | Erythematous crusted papulovesicles on the face | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67+ a few, LMP-1−, EBV ISH+, TCR gene rearrangement− | ND | Positive | None | Observation | Remission | 36 | AWD |
| 179 | F | 28 (26) | Erythematous crusted papulovesicles on the face and facial swelling | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67 15%, EBV ISH+, TCR gamma gene rearrangement− | ND | ND | None | Systemic steroid | Partial remission and recurrence | 36 | Death |
| 189 | M | 29 (25) | Erythematous crusted papulovesicles on the face, arm, and trunk | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67+ (< 5%), EBV ISH+, TCR gamma gene rearrangement− | ND | Positive | None | Systemic steroid | Partial remission and recurrence | 36 | AWD |
| 19 | F | 30 (27) | Erythematous crusted papulovesicles on the face and oral ulcer | CD3+, CD45RO+, CD20+ a few, CD56−, LMP-1−, Ki-67 < 5%, EBV ISH+, TCR gamma gene rearrangement+ | ND | ND | Cervical and axillary lymph node | Chemotherapy | Progressed to or accompanied extranodal NK/T-cell lymphoma, nasal type | 12 | AWD |
| 20 | M | 32 (15) | Erythematous crusted papulovesicles on the face and oral ulcer | CD3+, CD20+ a few, CD56−, granzyme B+, EBV ISH+, Ki-67 20% | 54,275 | ND | Palate, bone marrow | Chemotherapy, daratumumab | Progressed to extranodal NK/T-cell lymphoma, nasal type | 48 | AWD |
| 2111 | F | 33 (30) | Erythematous crusted papulovesicles on the face | CD3+, CD45RO+, CD20−, CD30−, CD56−, Ki-67 20%, EBV ISH+, TCR gamma gene rearrangement− | ND | Positive | Cervical lymph node, stomach | Chemotherapy | Progressed to Systemic EBV+ T-cell lymphoma | 18 | AWD |
| 2211 | M | 34 (33) | Erythematous crusted papulovesicles on the face, arm, and trunk, and oral ulcer | CD3+, CD45RO+, CD20−, CD30−, CD56−, Ki-67 20%, EBV ISH+, TCR gamma gene rearrangement+ | ND | ND | Bone marrow | Chemotherapy | Progressed to Systemic EBV+ T-cell lymphoma | 24 | AWD |
| 239 | M | 37 (32) | Erythematous crusted papulovesicles on the face, arm, and trunk | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67+ (< 5%), EBV ISH+, TCR gamma gene rearrangement− | ND | ND | Cervical lymph node | Chemotherapy | Partial remission and recurrence | 36 | AWD |
| 24 | M | 38 (38) | Erythematous crusted papulovesicles on the face | CD3+, CD4+ focal, CD8+ focal, CD20−, CD56−, granzyme B+ focal, Ki-67+, EBV ISH+, TCR gamma gene rearrangement+ | 925,529 | ND | Lung (Hodgkin lymphoma), bone marrow, spleen, bone | Systemic steroid → chemotherapy | Progressed to EBV+ Hodgkin lymphoma | 96 | AWD |
| 259 | F | 39 (36) | Erythematous crusted papulovesicles on the face, arm, and trunk | CD3+, CD45RO+, CD20+ a few, CD56−, Ki-67+ (< 5%), EBV ISH+, TCR gamma gene rearrangement− | ND | Positive | Cervical lymph node | Chemotherapy | Partial remission and recurrence | 36 | AWD |
| 2612 | F | 70 (70) | Erythematous crusted papulovesicles on the face, oral ulcer, and facial swelling | CD2+, CD3+, CD4+ a few, CD5+, CD8+ focal, CD20−, CD30−, CD56−, EBV ISH | 200,500 | Negative | None | Topical steroid | Partial remission and recurrence | 12 | AWD |
AWD alive with the disease, DOD died of disease, EBER-1 ISH EBV-encoded small nonpolyadenylated RNA (EBER)-1 by in situ hybridization, F female, IHC immunohistochemistry, M male, ND not done, NS not specified exact follow-up duration.