“…in 1972. Since then, similar cases have been described, and different treatments have been used to control the cutaneous lesions, such as corticosteroids, 3,4 dapsone, 5–8 cyclophosphamide, 9,10 colchicine, 11 and doxycycline 12 , with variable results. The most direct therapeutic approach is the use of either a purified concentrate of α 1 ‐AT (Prolastin®) 1,7,13–16 or plasma exchange therapy (PET), which restore plasma and tissue enzymatic concentrations to levels that prevent the progression of the destructive process; however, the high cost and limited availability of Prolastin® makes the use of this drug difficult.…”