Refractory linear IgA bullous dermatosis successfully treated with mycophenolate sodium

J Dermatolog Treat. 2008;19(6):364-7. doi: 10.1080/09546630801958246.

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).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Dermatologic Agents / immunology
  • Dermatologic Agents / therapeutic use*
  • Female
  • Humans
  • Immunoglobulin A / drug effects*
  • Immunosuppressive Agents / immunology
  • Immunosuppressive Agents / therapeutic use*
  • Mycophenolic Acid / analogs & derivatives*
  • Mycophenolic Acid / therapeutic use
  • Skin Diseases, Vesiculobullous / drug therapy*
  • Skin Diseases, Vesiculobullous / immunology
  • Treatment Outcome

Substances

  • Dermatologic Agents
  • Immunoglobulin A
  • Immunosuppressive Agents
  • Mycophenolic Acid