Appeal type: Experimental/Investigational
Decision: Overturned

Diagnosis: Pyoderma gangrenosum and pemphigus
Treatment: Intravenous Immune Globulin (J1561)
The insurer denied the Intravenous Immune Globulin (J1561).
The denial is overturned.

The patient is an adult male with severe, refractory pyoderma gangrenosum (PG) that has failed high-dose systemic steroids and systemic cyclosporine. He is developing new lesions and the old ones are enlarging. In the past, the severity of his disease required a below-knee amputation. He has received intravenous immunoglobulins in the past with improvement. This is a request for treatment with intravenous immunoglobulin (IVIG).

The health plan should cover the proposed treatment.

The patient has severe, refractory PG that has failed first and second-line treatment. Therefore, the use of IVIG is appropriate. 

The requested health service or treatment is more beneficial than any standard treatment for the insured's condition or disease. PG is an uncommon inflammatory and ulcerative skin disorder. The most common presentation of PG is the rapid development of one or more painful, purulent ulcers with undermined borders on sites of normal or traumatized skin.

Although multiple local and systemic therapies have been utilized for PG, high-quality efficacy studies are lacking for most interventions. As a result of the paucity of data on interventions for PG, definitive guidelines for patient management are lacking. The approach to the treatment of PG is primarily guided by small uncontrolled studies and clinical experience. In general, patients are managed with a combination of topical and/or systemic therapies that suppress the inflammatory process and wound care measures that optimize the environment for wound healing. Although initial signs of improvement may be evident within days of the start of treatment, weeks to months are often required to achieve complete ulcer healing. Most patients with PG cannot be managed with local therapy alone. Systemic treatment is used as a first-line intervention in PG that is not limited to a few superficial ulcers or a solitary plaque of vegetative PG. The systemic therapies utilized in PG are immunosuppressive and immunomodulatory agents that calm the inflammatory process to allow for wound healing. Systemic glucocorticoids often induce a rapid response and are typically used as initial therapy. In patients who cannot tolerate or fail to respond to systemic glucocorticoids, treatment with systemic cyclosporine can be attempted. Of note, topical or intralesional corticosteroids and topical tacrolimus are often used as adjuncts to systemic therapy. IVIG and the alkylating agents of cyclophosphamide and chlorambucil are options for patients with severe PG that is refractory to first- and second-line agents. IVIG is typically given in divided doses over the course of two to five days, repeated monthly. Efficacy data for IVIG for PG are limited. A systematic review of cases and case series documenting treatment of refractory pyoderma gangrenosum with IVIG (often in conjunction with systemic glucocorticoids) found complete responses in 26 of 49 patients (53 percent) and complete or partial responses in 43 of 49 patients (88 percent). The mean time to initial response was 3.5 weeks, and patients were treated for an average of 5.9 months. This patient has severe PG that has failed to respond to high-dose systemic steroids and cyclosporine. He has received IVIG in the past with improvement. Therefore, it is medically appropriate and medically necessary.