title: Dermatopathic lymphadenitis with generalized erythroderma in a patient with epstein-barr virus-associated hemophagocytic lymphohistiocytosis.
abstract: Here, we describe a patient with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) who simultaneously presented with generalized erythroderma and dermatopathic lymphadenitis (DL). A 63-year-old Korean woman presented at our hospital with fever, hepatosplenomegaly, axillary lymphadenopathy, and generalized erythrodermic eruption. The bone marrow biopsy findings were consistent with the diagnosis of HLH, and EBV DNA was detected using the polymerase chain reaction. Based on serologic tests that indicated a primary EBV infection, the patient was diagnosed with EBV-associated HLH. Histopathologic analysis of enlarged lymph nodes was consistent with DL, and EBV-encoded small nuclear RNA-positive mononuclear cells were detected. We assume that activated histiocytes, lymphocytes, and proinflammatory cytokines in HLH may have important roles in the development of generalized erythroderma and DL. Disrupted epidermal/dermal junctions owing to erythroderma may also be involved in the development of DL.
authors.affiliation: Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Woo Jin
authors.initials: WJ
authors.lastname: Lee
authors.forename: Deok Woo
authors.initials: DW
authors.lastname: Lee
authors.forename: Chae Hwa
authors.initials: CH
authors.lastname: Kim
authors.forename: Chong Hyun
authors.initials: CH
authors.lastname: Won
authors.forename: Sung Eun
authors.initials: SE
authors.lastname: Chang
authors.forename: Mi Woo
authors.initials: MW
authors.lastname: Lee
authors.forename: Jee Ho
authors.initials: JH
authors.lastname: Choi
authors.forename: Kee Chan
authors.initials: KC
authors.lastname: Moon
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016572:Cyclosporine / Q000008:administration & dosage; D003873:Dermatitis, Exfoliative / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D003907:Dexamethasone / Q000627:therapeutic use; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000473:pathology; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008199:Lymphadenitis / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D008875:Middle Aged
chemical_list: D000893:Anti-Inflammatory Agents; D005047:Etoposide; D003907:Dexamethasone; D016572:Cyclosporine