Refractory macrophage activation syndrome in the setting of adult-onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus

J Cutan Pathol. 2019 Jul;46(7):528-531. doi: 10.1111/cup.13466. Epub 2019 Apr 23.

Abstract

A 19-year-old Caucasian female with adult-onset Still 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.

Keywords: adult-onset Still disease; canakinumab; hemophagocytic lymphohistiocytosis; histopathology; macrophage activation syndrome; skin biopsy; tacrolimus.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Erythrocytes / metabolism
  • Erythrocytes / pathology
  • Female
  • Histiocytes / metabolism
  • Histiocytes / pathology
  • Humans
  • Lower Extremity / pathology
  • Lymphocytes / metabolism
  • Lymphocytes / pathology
  • Lymphohistiocytosis, Hemophagocytic* / diagnosis
  • Lymphohistiocytosis, Hemophagocytic* / metabolism
  • Lymphohistiocytosis, Hemophagocytic* / pathology
  • Macrophage Activation Syndrome* / diagnosis
  • Macrophage Activation Syndrome* / metabolism
  • Macrophage Activation Syndrome* / pathology
  • Still's Disease, Adult-Onset* / diagnosis
  • Still's Disease, Adult-Onset* / metabolism
  • Still's Disease, Adult-Onset* / pathology