A Case of Hydroa Vacciniforme-Like Lymphoproliferative Disorder Presenting As Orogenital Ulcerations

Brief Report
Brief Report Annals of Dermatology 2022

2022;;34
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https://doi.org/10.5021/ad.20.199
A Case of Hydroa Vacciniforme-Like Lymphoproliferative

Disorder Presenting As Orogenital Ulcerations
Yingyi Li1,2,3, Yang Wang1,2,3
1
Department of Dermatology and Venerology, Peking University First Hospital, 2Beijing Key Laboratory of Molecular Diagnosis on
Dermatoses, 3National Clinical Research Center for Skin and Immune Diseases, Beijing, China
Dear Editor: constitutional symptoms. He was diagnosed with classic HV

Hydroa vacciniforme (HV)-like lymphoproliferative disease at age 2 years, based on the typical papulovesicular eruptions
(LPD) is associated with chronic active Epstein-Barr virus on his face and photosensitivity. The symptoms spontane-
(CAEBV) infection. It ranges from classic and severe/systemic ously resolved 2 years before without recurrence, leaving facial
HV to HV-like lymphoma (HVLL)1. Cutaneous manifesta- varioliform scars (Fig. 1A). Physical examination revealed
tions include vesiculopapules, bullae, ulcers, and facial swell- marked swelling on the lower lip and massive erosions with
ing1. HVLL usually has a long clinical course with sponta- ulcerations on the scrotum (Fig. 1A, B). No lymphadenopathy
neous resolution1 but may progress to more severe and life- was observed. A skin biopsy from the lower lip showed diffuse
threatening conditions2. We report a rare case of HV-like LPD infiltrates of atypical lymphoid cells throughout the dermis,
that evolved from classic HV to orogenital ulcerations. with remarkable epidermotropism (Fig. 2A). Immunohisto-
A 17-year-old Chinese boy presented with a 2-month histo- chemical analysis revealed CD3+++, CD4+++, Granzyme
ry of painful orogenital swelling and ulcerative lesions without B++, and TIA1+++ infiltrating lymphocytes, consistent with
Fig. 1. We received the patient’s

consent form about publishing all
photographic materials. (A) Lower lip
swelling with scales and varioliform
A B scars on the face. (B) Remarkable ero-
sions and ulcerations on the scrotum.
Received July 21, 2020 Revised December 14, 2020 Accepted January 19, 2021
Corresponding Author
Yang Wang
Department of Dermatology and Venerology, Peking University First Hospital, No.8 Xishiku Street, Xicheng District, Beijing 100034, China
Tel: +86-10-83572350, Fax: +86-10-66551216, E-mail: [email protected]
https://orcid.org/0000-0001-7805-2861
https://doi.org/10.5021/ad.20.199 389
Brief Report
A B
H&E 50 200 CD4 50 200
C D
GB 50 200 TIA-1 50 200
EBER 50 200
Fig. 2. (A) Histopathology shows diffuse infiltrate of atypical lymphoid cells in the dermis, with marked epidermotropism (H&E, left:
magnification ×50, right: magnification ×200). (B~D) Immunostaining reveals that the infiltrating lymphocytes were positive for CD4,
Granzyme B, and TIA-1 (left: magnification ×50; right: magnification ×200). (E) In situ hybridization showed strong EBV-encoded small
RNA (EBER) positivity throughout the infiltrating lymphocytes (left: magnification ×50; right: magnification ×200).
an activated cytotoxic T-cell phenotype (Fig. 2B~D). In situ patient in childhood. As the disease progresses, more exten-
hybridization for EBV-encoded small RNA (EBER) revealed sive skin lesions develop1, such as blisters, ulcers, exaggerated
diffuse positivity (Fig. 2E). T-cell receptor gene gamma rear- arthropod bite reactions, and edema4. Oculomucosal and gas-
rangement indicated clonality. Peripheral blood flow cytome- trointestinal involvement are sometimes complicated5. Overt
try demonstrated normal amounts of CD3+/CD4+ T cells and systemic lymphoma may occur and lead to poor prognosis1,4.
CD3+CD8+ T cells. The EBV DNA viral load in peripheral No standard treatment has been established. A conservative
lymphocytes was elevated (1.69×106 copies/ml; normal range, approach is recommended for indolent cases, whereas hema-
0~5,000 copies/ml). Titers of IgG antibodies to the EBV capsid topoietic stem cell transplantation might benefit patients with
antigen and EBV nuclear antigen-1 were high. These findings advanced cases1,2.
confirmed the diagnosis of HV-like LPD. The patient was Our patient presented with ulcerative lesions solely on the
then treated with interferon alpha-2b 300 IU 3 times per week orogenital area after 2 years of HV remission, which has not
based on previous reports but showed no significant improve- been described in previous cases of HV-like LPD. The pres-
ment2. As his orogenital swelling and ulcerations worsened, ence of clonal T cells indicated the possibility of progression
human leukocyte antigen-haploidentical donor lymphocyte to lymphoma2. Conservative treatment was ineffective for dis-
infusion was administered 3. A total of 4.8×10 9 peripheral ease control. This case highlights the importance of recogniz-
blood mononuclear cells (about 1×108/kg) was infused. The le- ing the uncommon clinical features of CAEBV. It shows that
sions gradually resolved and remained stable thereafter. classic HV may progress even after years of remission of the
HV-like LPD is a chronic EBV-positive lymphoproliferative initial manifestations.
disorder with a broad spectrum of clinical aggressive1,2. Clas-
sic HV is a benign photodermatosis characterized by cursted
papulovesicles and varicelliform scars after healing1, as in our
390
Brief Report
ACKNOWLEDGMENT REFERENCES
The authors thank the staff of Department of Hematology at 1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et
Peking University First Hospital for providing treatment in- al. WHO classification of tumours of haematopoietic and lymphoid
formation. tissues. 4th revised ed. Lyon: International Agency for Research on
Cancer, 2017:355-362.
CONFLICTS OF INTEREST 2. Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M,
Durán-McKinster C, Ruiz-Maldonado R, et al. Hydroa vaccin-
The authors have nothing to disclose. iforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder
with risk to develop a systemic lymphoma. Blood 2013;122:3101-3110.
FUNDING SOURCE 3. Wang Q, Liu H, Zhang X, Liu Q, Xing Y, Zhou X, et al. High doses of
mother’s lymphocyte infusion to treat EBV-positive T-cell lympho-
None. proliferative disorders in childhood. Blood 2010;116:5941-5947.
4. Sangueza M, Plaza JA. Hydroa vacciniforme-like cutaneous T-cell
ORCID lymphoma: clinicopathologic and immunohistochemical study of
12 cases. J Am Acad Dermatol 2013;69:112-119.
Yingyi Li, https://orcid.org/0000-0003-1097-0230 5. Yamamoto T, Hirai Y, Miyake T, Yamasaki O, Morizane S, Iwatsuki K.
Yang Wang, https://orcid.org/0000-0001-7805-2861 Oculomucosal and gastrointestinal involvement in Epstein-Barr virus-
associated hydroa vacciniforme. Eur J Dermatol 2012;22:380-383.
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Brief Report

Brief Report Annals of Dermatology 2022

A Case of Hydroa Vacciniforme-Like Lymphoproliferative

Dear Editor: constitutional symptoms. He was diagnosed with classic HV

Fig. 1. We received the patient’s

H&E 50 200 CD4 50 200

GB 50 200 TIA-1 50 200

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