Hemophagocytic lymphohistiocytosis syndrome secondary to systemic hydroa vacciniforme lymphoproliferative disorder: a case report and literature review

• Published in: Pifu-xingbing zhenliaoxue zazhi Vol. 31; no. 2; pp. 102 - 108
• Main Authors: Sai YANG, Mohammad Hassan HEIDARI, Ruzeng XUE, Ying LUO, Rongyi CHEN, Yongfeng CHEN, Yunsheng LIANG
• Format: Journal Article
• Language: Chinese
• Published: editoiral office of Journal of Diagnosis and Therapy on Dermato-venereology 01.02.2024
• Subjects: ebv; hemophagocytic lymphohistiocytosis syndrome; hydroa vacciniforme lymphoproliferative disorder
• ISSN: 1674-8468
• DOI: 10.3969/j.issn.1674-8468.2024.02.006

Objective To report a case of hemophagocytic lymphohistiocytosis syndrome (HLH) secondary to systemic hydroa vacciniforme lymphoproliferative disorder (systemic HVLPD) and review relevant literature, in order to improve understanding of the disease. Methods Clinical data of the case, laboratory examination, histopathology, immunohistochemistry, TCR gene rearrangement, and whole exon gene sequencing were collected for analyses. Results A 19 years old man was admitted to the hospital due to generalized papules, blisters, necrotic scabs and fever for 7 months. The clinical manifestations included hydroa vacciniforme-like rashes, lymphadenopathy, splenomegaly, pancytopenia, hypertriglyceridemia, decreased NK cell activity, high levels of sIL-2r, positive for VCA-IgA and EA-IgA, and elevated EBV DNA load. The whole exon sequencing did not show pathogenic gene mutations. Histopathological changes in the skin lesions included liquefaction degeneration of epidermal and follicular basal cells, pigmentary incontinence,