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

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. K E Y W O R D Sadult-onset Still disease, canakinumab, hemophagocytic lymphohistiocytosis, histopathology, macrophage activation syndrome, skin biopsy, tacrolimus

Lessons from dermatology about inflammatory responses in Covid‐19

“…However, other authors described polymorphous cutaneous lesions or atypical cutaneous presentations as erythematous or brown eruptions, 59 less commonly violaceous in colour, 59 linear configurations simulating dermographism 60 or flagellate dermatitis, prurigo‐pigmentosa‐like, 59 urticarial papules, 60 dermatomyositis‐like 59 and lichen amyloidosis‐like eruptions 59 . Chamseddin et al 61 reported a 19‐year‐old woman who developed acute clinical decompensation and atypical annular papules and plaques with purpura on the down extremities. A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH).…”

“…A punch biopsy demonstrated histiocytes with engulfed degenerated erythrocytes and lymphocytes, consistent with hemophagocytic lymphohistiocytosis (HLH). MAS, a variant of secondary HLH occurring in the setting of autoimmune disease, is a life‐threatening condition with reported mortality rate of 20% to 50% 61 . It has been reported in 3% to 20% of AOSD cases 61 .…”

“…MAS, a variant of secondary HLH occurring in the setting of autoimmune disease, is a life‐threatening condition with reported mortality rate of 20% to 50% 61 . It has been reported in 3% to 20% of AOSD cases 61 . MAS is thought to occur because of widespread immune activation with proliferation of cytotoxic CD8+ T‐cells and macrophages which develop into hemophagocytosis and cytotoxic storm 61 .…”

“…It has been reported in 3% to 20% of AOSD cases 61 . MAS is thought to occur because of widespread immune activation with proliferation of cytotoxic CD8+ T‐cells and macrophages which develop into hemophagocytosis and cytotoxic storm 61 . Three cases of MAS secondary to SLE or dermatomyositis have reported skin biopsies with similar histologic findings 61 .…”

“…MAS is thought to occur because of widespread immune activation with proliferation of cytotoxic CD8+ T‐cells and macrophages which develop into hemophagocytosis and cytotoxic storm 61 . Three cases of MAS secondary to SLE or dermatomyositis have reported skin biopsies with similar histologic findings 61 . Cutaneous hemophagocytosis does not always indicate systemic MAS and may be incidentally found in leukocytoclastic vasculitis, lupus erythematous, arthropod bites, erysipelas, acne conglobata, and Sweet syndrome 61 …”