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Refractory macrophage activation syndrome in the setting of adult‐onset Still disease with hemophagocytic lymphohistiocytosis detected on skin biopsy treated with canakinumab and tacrolimus
Author(s) -
Chamseddin Bahir,
Marks Etan,
Dominguez Arturo,
Wysocki Christian,
Vandergriff Travis
Publication year - 2019
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.13466
Subject(s) - macrophage activation syndrome , medicine , hemophagocytic lymphohistiocytosis , pathology , histiocyte , hemophagocytosis , skin biopsy , histiocytosis , tacrolimus , biopsy , adult onset still's disease , refractory (planetary science) , dermatology , disease , transplantation , bone marrow , physics , pancytopenia , astrobiology
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.