title: Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma.
abstract: Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. We are reporting 7 cases of an unusual clinical variant of HVL with primarily periorbital edema. All of our patients in this series presented with progressive periorbital edema that was accompanied with systemic symptoms including fever, malaise, and lymphadenopathy. Most cases were initially misinterpreted as inflammatory processes including cellulitis, arthropod bite reactions, and periorbital lupus erythematosus. The biopsy of these lesions revealed an atypical lymphocytic infiltrate predominantly distributed in the deep dermis and in subcutaneous fat. Immunohistochemistry studies revealed a cytotoxic T-cell (CD8) profile. All cases were associated with Epstein-Barr virus infection. Our study presents a rare clinical variant of HVL with predominant periorbital edema. This variant could potentially be overlooked and misdiagnosed as an inflammatory condition; thus, it needs to be included in the differential diagnosis of periorbital edema in young patients.
authors.affiliation: *Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, WI; and †Department of Pathology and Dermatology, Division of Dermatopathology, Hospital Obrero, La Paz, Bolivia.
authors.forename: Jose A
authors.initials: JA
authors.lastname: Plaza
authors.forename: Martin
authors.initials: M
authors.lastname: Sangueza
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002648:Child; D003937:Diagnosis, Differential; D004487:Edema / Q000209:etiology / Q000473:pathology* / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology / Q000628:therapy / Q000821:virology; D005134:Eye Neoplasms / Q000737:chemistry / Q000145:classification / Q000473:pathology* / Q000628:therapy / Q000821:virology; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D006837:Hydroa Vacciniforme / Q000145:classification / Q000473:pathology* / Q000628:therapy / Q000821:virology; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D016410:Lymphoma, T-Cell, Cutaneous / Q000737:chemistry / Q000145:classification / Q000473:pathology* / Q000628:therapy / Q000821:virology; D008297:Male; D011237:Predictive Value of Tests; D012367:RNA, Viral / Q000235:genetics; D012878:Skin Neoplasms / Q000737:chemistry / Q000145:classification / Q000473:pathology* / Q000628:therapy / Q000821:virology
chemical_list: D014408:Biomarkers, Tumor; C099537:Epstein-Barr virus encoded RNA 1; D012367:RNA, Viral