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Dermatopathic Lymphadenitis With Generalized Erythroderma in a Patient With Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis

Lee, Woo Jin MD; Lee, Deok Woo MD; Kim, Chae Hwa MD; Won, Chong Hyun MD; Chang, Sung Eun MD; Lee, Mi Woo MD; Choi, Jee Ho MD; Moon, Kee Chan MD

American Journal of Dermatopathology: June 2010 - Volume 32 - Issue 4 - pp 357-361
doi: 10.1097/DAD.0b013e3181b2a50f
Brief Report

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.

From the Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Funding sources: none.

Conflict of Interest Disclosure: none declared.

Reprints: Mi Woo Lee, MD, Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, 388-1 Pungnap dong, Songpa-gu, Seoul 138-736, Korea (e-mail: jijulee@hanmail.net).

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