Management

Phemphigus is almost always fatal if treatment is not initiated (1).

The basis of treatment is to suppress the immune system in order to blunt the autoimmune response (2).

Systemic steroids are the usual treatment. The exact dose depends upon the patient's size and the disease severity; a common dose may be 60-100 mg prednisolone per day. The dose should be reduced immediately that the disease is controlled for example, by 20-30 mg/week. An alternate day regime may be advised in order to minimise side effects. Steroid sparing immunosuppressives (e.g. cyclophosphamide or azathioprine, plasmaphoresis) are often included. Potent topical steroids e.g. clobetasol propionate may be sufficient in mild cases (3).

Topical treatment is symptomatic to prevent secondary infection and to protect raw skin (3).

Study evidence showed that rituximab was superior to mycophenolate mofetil in producing sustained complete remission at 52 weeks in patients with pemphigus vulgaris (4)

• rituximab resulted in a greater reduction in glucocorticoid use than mycophenolate mofetil, but more patients in the rituximab group had serious adverse events (4)

Mortality is high - 15-25% - and may be due to the side effects of treatment e.g. osteoporosis, immunosuppression.

Reference:

• 1. Bystryn J, Rudolph J.L. Pemphigus. The Lancet 2005; 336(9479): 61-73
• 2. Stanley J.R, Amagai M. Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded-Skin Syndrome. NEJM 2006; 355:1800-1810
• 3. Harman K.E et al. Guidelines for the management of pemphigus vulgaris. BJD 2003; 149: 926-937
• 4. Werth VP et al. Rituximab versus Mycophenolate Mofetil in Patients with Pemphigus Vulgaris.NEJM May 19, 2021