Chronic Active Epstein-Barr Virus Infection-Associated Hydroa Vacciniforme-Like Eruption and Behçet's-Like Orogenital Ulcers

Abstract

The cutaneous manifestations of chronic active Epstein-Barr virus (EBV) infection can be diverse. Among them, hydroa vacciniforme-like eruption is one of the best-known features. Although rare, mucosal ulcers have been reported to be associated with EBV as a result of primary infection or immune suppression. We describe a 65-year-old female with recurrent necrotic papulovesicles on the face and both arms for 2 years. She also complained of recurrent oral and genital mucosal ulcers developing simultaneously with skin eruptions. They appeared periodically during the spring and summer and were triggered or aggravated by sun exposure. Skin biopsies from the face and genitalia showed identical findings with dense lymphocytic infiltrations. In addition, in situ hybridization revealed EBV-positive lymphoid cells in both specimens. To our knowledge, this is the first case of serologically and pathologically proven chronic active EBV infection presenting hydroa vacciniforme-like eruption and orogenital ulcers at the same time in one patient.

CD30-Positive T-Cell Lymphoproliferative Disease of the Oral Mucosa in Children: A Manifestation of Epstein-Barr Virus-Associated T-Lymphoproliferative Disorder

“…Usually, systemic symptoms such as fever persist for more than six months with increased EB viral load in blood or tissues. Although the most frequent symptoms include organomegaly, anemia, and thrombocytopenia, patients often present with uncommon clinical findings such as coronary aneurysms, central nervous system involvement, bowel perforation, or Behçet-like orogenital ulcer [ 16 ]. The clinical course is variable and depends on the immune response of the individuals.…”

Atypical hydroa vacciniforme mimicking dermatomyositis: A case report in a Lebanese girl

“…Atypical hydroa vacciniforme (HV) is a rare vesiculopapular eruption reported mainly in patients from Asia and Latin America 1 , 2 , 3 and presents with similar but more severe cutaneous manifestations than classic HV. In addition, systemic findings may be present.…”

“…In addition, systemic findings may be present. 1 Distinguishing between classic and atypical HV is essential, as the latter carries a worse prognosis and may progress to HV-like lymphoma. 1 , 2 Involvement of muscle resulting in a picture similar to dermatomyositis (DM) is a rarely reported presentation of atypical HV.…”

Hydroa Vacciniforme-Like Eruption Associated with Epstein-Barr Virus Infection in an Older Adult

“…It is characterized by an edematous, crusted papulovesicular eruption similar to HV 1 2 3 4 . Unlike HV, however, it could also occur in non-sun-exposed areas, and is accompanied by fever, lymphadenopathy, or hepatosplenomegaly 1 2 3 4 . Because HV-like lymphoma could occur in non-sun-exposed areas and is accompanied by systemic symptoms, it can be confused with HV-like eruption.…”

“…These three disease categories were related to EBV infection and predominantly occur in children and adolescents. However, HV-like eruption is occasionally observed in adults aged older than 60 years: one case was that of a 65-year-old woman with orogenital ulcer, whose symptoms were well controlled with corticosteroid and famciclovir 3 , whereas the other was a case of a 74-year-old man whose condition deteriorated rapidly and who died within 3 months of diagnosis 4 . Thus, we report a case of HV-like eruption in an older adult manifesting chronic feature.…”