CMS Pub. 100-03, ch. 1, § 250.3

Mucocutaneous Blistering Diseases

(Rev. 173, Issued: 09-04-14, Effective: Upon Implementation: of ICD-10, Implementation: Upon Implementation of ICD-10)

Intravenous immune globulin (IVIg) is a blood product prepared from the pooled plasma of

donors. It has been used to treat a variety of autoimmune diseases, including mucocutaneous

blistering diseases. It has fewer side effects than steroids or immunosuppressive agents.

Effective October 1, 2002, IVIg is covered for the treatment of biopsy-proven: (1) Pemphigus

Vulgaris, (2) Pemphigus Foliaceus, (3) Bullous Pemphigoid, (4) Mucous Membrane Pemphigoid

(a.k.a., Cicatricial Pemphigoid), and, (5) Epidermolysis Bullosa Acquisita for the following

patient subpopulations:

• Patients who have failed conventional therapy. A/B MACs (A) and (B) have the

discretion to define what constitutes failure of conventional therapy;

• Patients in whom conventional therapy is otherwise contraindicated. conventional

therapy; or

• Patients with rapidly progressive disease in whom a clinical response could not be

affected quickly enough using conventional agents. In such situations IVIg therapy would be

given along with conventional treatment(s) and the IVIg would be used only until the

conventional therapy could take effect.

In addition, IVIg for the treatment of autoimmune mucocutaneous blistering diseases must be

used only for short-term therapy and not as a maintenance therapy. A/B MACs (A) and (B) have

the discretion to decide what constitutes short-term therapy.