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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.

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This article was published in the following journal.

Name: Journal of cutaneous pathology
ISSN: 1600-0560
Pages:

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