Phu, 27, endured over a year of debilitating pemphigus, an autoimmune disease that caused severe blistering across his mucous membranes and skin. Despite initial poor responses to various treatments, a specialized medical team at Tam Anh General Hospital, Ho Chi Minh City, successfully managed his refractory condition. After nearly two months of an advanced therapeutic regimen, new lesions ceased appearing, and his skin began to recover, allowing him to regain some normalcy.

Phu's ordeal began with small, recurring sores in his mouth. Within about one month, numerous erosions and fluid-filled blisters, resembling burns and measuring a few millimeters to several centimeters, appeared on his face, neck, and hands. These thin, fragile blisters easily ruptured, leaving red, painful, oozing areas that would then scab over, with new outbreaks appearing continuously.

Diagnosed with pemphigus, a rare autoimmune blistering disease affecting approximately 2.8 out of 1,000,000 people, Phu received various treatments, including corticosteroids, antibiotics, and immunosuppressants. However, his body responded poorly, and his condition worsened. Doctor Nguyen Phuc Tan, from the Clinical Immunology Department at Tam Anh General Hospital, Ho Chi Minh City, explained that pemphigus causes the immune system to attack the cells binding skin and mucous membranes, leading to fragile, blister-like lesions. Phu’s condition progressed rapidly and severely, with widespread lesions and superinfection, posing a high risk of systemic sepsis complications.

Doctor Tan consults Phu during a follow-up. *Photo: Tam Anh General Hospital*

Doctor Tan’s initial treatment regimen, aimed at drying the ulcers and controlling the overactive immune system, proved ineffective. The lesions continued to spread, and the superinfection worsened, leading the Clinical Immunology Department to diagnose Phu with refractory pemphigus. A critical challenge arose with rituximab, a biologic drug fundamental for moderate to severe pemphigus that selectively inhibits B cells, which produce disease-causing autoantibodies. Phu’s active infection upon admission meant immediate use of such a strong immunosuppressive biologic drug could severely worsen the infection risk.

To navigate this dilemma, doctors opted for intravenous immunoglobulin (IVIg) as a "bridge" therapy. This approach aimed to modulate abnormal immune activity and help control the disease without further deep immunosuppression. Concurrently, Phu received aggressive infection control and intensive skin care.

Following the initial intravenous immunoglobulin cycles, the number of new blisters gradually decreased, lesions dried, and inflammation was better controlled. Once the infection stabilized, the team initiated two courses of rituximab according to the standard protocol to directly target the disease mechanism. This phased combination treatment gradually brought the disease under control. New blisters stopped appearing, eroded skin healed, and Phu’s body progressively recovered. After nearly two months, his skin showed no new lesions, with only post-inflammatory hyperpigmentation remaining where large ulcerated areas once covered his body.

According to Doctor Tan, while there is currently no complete cure for pemphigus, long-term control is achievable with the correct treatment regimen and close monitoring. The disease is often missed in its early stages due to atypical initial symptoms, frequently mistaken for common ulcers. When patients seek hospital care late, prolonged immune dysregulation and widespread lesions make treatment more challenging.

Many pemphigus patients experience prolonged psychological trauma even after overcoming the critical phase. Phu, for instance, despite having no new skin lesions and only post-inflammatory hyperpigmentation, remains stressed, anxious, and suffers from insomnia due to fear of blister outbreaks. Doctors reassured him, clearly explaining the risks and preventive measures to reduce his stress and help him accept living with the disease. Currently, he continues regular follow-ups and maintains an appropriate immunosuppressant regimen to ensure stable disease control and prevent recurrence.

Minh Huong

*Patient's name has been changed.