​Saving a young man with a disease first discovered in Vietnam

The patient, from Thanh Hoa, began to have erosive ulcers on the oral mucosa and purple-red patches and scalds scattered all over his body in November last year. At the local hospital for examination, the doctor discovered a large tumor behind the peritoneum, then was transferred to the Central Dermatology Hospital, and finally to Bach Mai Hospital.

From the skin biopsy results, the doctor diagnosed him with toxic epidermal necrolysis. In January 2024, the patient was diagnosed with paraneoplastic pempigus – bilateral ureteral stones, and a weakened physical condition.

“Euraplastic pemphigus is a rare disease, not yet found in Vietnamese medical literature. In the world, the incidence of the disease is one in a million people,” said MSc. Tran Thi Quyen, Department of Dermatology, Bach Mai Hospital, said. afternoon of June 27.

The patient was scheduled for surgery, but his condition was weak so he was discharged from the hospital for nutritional supplementation. Since April, the patient has had new outbreaks with symptoms of fever, skin erosion, ulceration of the oral mucosa, requiring plasma replacement and antibiotics. At the end of May, he had surgery to remove the tumor to completely cure the disease. Currently, the male student has recovered and been discharged from the hospital.

According to Dr. Quyen, the treatment process for patients encountered many difficulties due to multi-morphological lesions and inconsistent histopathological results. Therefore, doctors had to continuously consult and simultaneously look up and study many medical literatures to make the most accurate diagnosis.

The care process was also very difficult because the patient had a poor response to conventional immunotherapy, losing 10 kg in a month. He often had sepsis and urinary tract infections caused by multi-resistant Pseudomonas aeruginosa, with a high mortality rate.

Associate Professor Dao Xuan Co, Director of Bach Mai Hospital, said there was a time when the family asked to take the patient home, but the doctors encouraged the relatives to try to keep the child in the hospital. At the end of treatment, the male student was paid nearly 700 million VND by Health Insurance, with his family paying more than 53 million VND.

Paraneoplastic pemphigus is a rare disease associated with lymphoproliferative disorders. It usually occurs in people between the ages of 45 and 70.