Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium

Abstract

Linear IgA bullous dermatosis (LABD) is a rare, blistering autoimmune disease characterized by linear deposits of IgA at the basement membrane zone (BMZ), with the possible presence of circulating IgA anti‐BMZ antibodies. LABD of childhood is usually self‐healing, while in adults it follows a more prolonged course and refractory cases may rarely occur. The first‐line treatment for LABD is dapsone in monotherapy or in combination with systemic corticosteroids, but various therapeutic approaches have been used in non‐responder patients. We report two adult patients with refractory LABD successfully treated with enteric‐coated mycophenolate sodium (EC‐MPS), a recently introduced formulation of mycophenolic acid (MPA). MPA is an immunosuppressive agent that acts by inhibiting monophosphate dehydrogenase, a key enzyme in the novo synthesis of purines. Based on the present cases, we indicate EC‐MPS as being a safe and effective adjuvant therapy in the treatment of LABD when dapsone or the other steroid‐sparing drugs fail. It seems to offer an improved gastric side effect profile in comparison with the classic formulation of MPA, namely its ester mycophenolate mofetil (MMF).

• Linear IgA bullous dermatosis
• mycophenolate mofetil
• mycophenolate sodium
• mycophenolic acid