Refractory Macrophage Activation Syndrome in the setting of Adult Onset Still's Disease with Hemophagocytic Lymphohistiocytosis Detected on Skin Biopsy treated with Canakinumab and Tacrolimus.

08:00 EDT 30th March 2019 | BioPortfolio

Summary of "Refractory Macrophage Activation Syndrome in the setting of Adult Onset Still's Disease with Hemophagocytic Lymphohistiocytosis Detected on Skin Biopsy treated with Canakinumab and Tacrolimus."

A 19-year-old Caucasian female with adult onset Still's disease (AOSD) presented for evaluation of an acute clinical decompensation and atypical annular papules and plaques with purpura on the lower extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). HLH, clinically referred to as macrophage activation syndrome, is a rare complication of AOSD and is life threatening. Relevant clinical, laboratory, and histologic features of this diagnosis are reviewed. This article is protected by copyright. All rights reserved.


Journal Details

This article was published in the following journal.

Name: Journal of cutaneous pathology
ISSN: 1600-0560


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