Korean J Dermatol.  2014 Jun;52(6):421-424.

Hemophagocytic Lymphohistiocytosis Manifesting as a Purpuric Patch

• Lyu SM
• Byun JY
• Choi YW
• Choi HY

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a fatal, systemic inflammatory syndrome that characteristically presents hemophagocytic histiocytes infiltrating into various organs. Common features of HLH are abrupt onset of high fever, malaise, lymphadenopathy, hepatosplenomegaly, and abnormal laboratory findings including pancytopenia, hypertriglyceridemia, hypofibrinogenemia, and high ferritin levels. Cutaneous manifestations, which occur in 6~65% of patients, can develop at any moment in the clinical course of the syndrome. Most skin eruptions in HLH patients are generalized, transient maculopapular rashes. We describe an HLH patient who first manifested cutaneous symptoms as a large purpuric patch on her thigh. A skin biopsy found high levels of hemophagocytic histiocytes.

Keyword

Hemophagocytic lymphohistiocytosis; Purpura

MeSH Terms

Biopsy
Exanthema
Ferritins
Fever
Histiocytes
Humans
Hypertriglyceridemia
Lymphatic Diseases
Lymphohistiocytosis, Hemophagocytic*
Pancytopenia
Purpura
Skin
Thigh
Ferritins