Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium.
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).
Marzano
Angelo V
AV
Institute of Dermatological Sciences, University of Milan, Fondazione IRCCS Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy. francesca.alessi@unimi.it
Ramoni
Stefano
S
Spinelli
Diana
D
Alessi
Elvio
E
Berti
Emilio
E
D000328:Adult; D003879:Dermatologic Agents / Q000276:immunology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000187:drug effects*; D007166:Immunosuppressive Agents / Q000276:immunology / Q000627:therapeutic use*; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy* / Q000276:immunology; D016896:Treatment Outcome
D003879:Dermatologic Agents; D007070:Immunoglobulin A; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid