The Department of Dermatology, Bach Mai Hospital has just successfully treated the 10th rare case in the world.
After nearly 200 days with 2 hospitalizations, patient Nguyen Quoc T. with paraneoplastic pemphigus and a large retroperitoneal tumor measuring about 136x65x131mm was saved and successfully treated.
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Although there were times when the patient and his family asked to follow God's will by "abandoning treatment and going home to wait for death", the doctors and medical staff of the Dermatology Department encouraged and did their best to help the patient "defy God's will" and return to life.
To learn about this rare miracle case, we had a talk with Dr. Tran Thai Son, Head of Dermatology Department, Bach Mai Hospital - a doctor with a warm heart who devoted his heart, effort, and money to help the 19-year-old man return to life.
BSCKII.Tran Thai Son, December 2023, patient Nguyen Quoc T., male, 19 years old, was admitted to the Dermatology Department, Bach Mai Hospital in a state of severe oral mucosal erosion, accompanied by diverse skin lesions with manifestations of papules, purple-red patches, blisters, and vesicles scattered all over the body. The skin lesions quickly progressed into large skin erosions, occupying > 70% of the body surface area.
The painful ulcerative lesions on the oral mucosa caused the patient to have a poor appetite and his health quickly deteriorated. In addition to the severe skin and mucosal manifestations, we also discovered that the patient had a large retroperitoneal tumor, measuring approximately 136x65x131mm, pressing on many surrounding organs.
Due to the diverse skin lesions, the patient was indicated for multiple skin biopsies. However, the biopsies gave different results: lichenoid drug reaction, erythema multiforme, toxic epidermal necrolysis.
Faced with the extremely rapid progression of skin lesions, with a high risk of affecting life, Bach Mai Hospital has organized hospital-wide consultations many times with the participation of many leading experts...
However, the clinical signs and complex test results, each time giving different results, made it impossible for the Bach Mai Hospital's Consultation Council to make a final conclusion for a suitable treatment plan.
We searched the literature again, found similar cases and made the final diagnosis. Patient Trinh had paraneoplastic pemphigus with evidence of positive IgG in the intercellular space and basement membrane by indirect immunofluorescence assay on mouse bladder.
Paraneoplastic pemphigus is a rare autoimmune skin disease involving tumors in the body. The tumors produce abnormal proteins that are targeted by the body's immune system, creating cross-immunity with proteins in the skin and mucous membranes.
The most common are lymphoproliferative or hematological malignancies. This is a very rare disease in Dermatology and there is very little data in the world to estimate its incidence.
Based on published reports, the estimated incidence of pemphigus is less than 1/1,000,000 population per year. In Vietnam, data on pemphigus is quite limited and no case reports have been published.
Finding out about Pemphigus spongiform encephalopathy has been difficult, but treating the disease is an extremely big challenge, requiring the coordination of many specialties and leading experts.
With a diagnosis of Pemphigus vulgaris, tumor treatment is one of the first steps to treat the disease. However, due to extensive skin lesions and a weakened physical condition, patient Trinh was not healthy enough to undergo major surgery. The hospital's consultation board agreed to treat the skin lesions and improve patient Trinh's physical condition before surgery.
In the early stages, the patient's lesions responded poorly to conventional immunosuppressive therapy. The skin erosions continued to spread to >80% of the body surface area, with severe damage to the mucous membranes of the eyes, ears, and mouth.
The patient quickly lost 10kg within 1 month. The patient only weighed more than 30kg, his body was so emaciated that only patches of skin remained clinging to the bones.
There was a time when the patient and his family gave up and expressed their wish to go home. As people who directly cared for, treated and cleaned the patient’s ulcerated skin every day, we could never forget the helpless and desperate look in the patient’s eyes as he whispered to us: “Doctor, when can I die?”
The question of a young man, not even as old as my son, made me determined to encourage the patient, the patient's family and the medical team of the department to work together to save the patient's life.
The patient was transferred to the Intensive Care Center and was prescribed plasma exchange combined with immunosuppressive therapy, the skin lesions gradually healed. Here, the treatment process for the patient continued to encounter many difficulties.
Patients with sepsis, urinary tract infection caused by multidrug-resistant Pseudomonas aeruginosa, anemia, electrolyte disturbances, etc. have a very high risk of death, over 90%. It must be said that the progression of patient Trinh is an extremely big challenge for us.
If it were an ordinary person, seeing the skin lesions all over the patient's body, they would probably run away from afar. We, the doctors and nurses, still diligently and meticulously cleaned the skin lesions and provided comprehensive care for patient Trinh.
When the skin lesions had healed >95% and the oral mucosa lesions had partially recovered, the patient quickly fell into a new outbreak with a more severe and complicated course than the previous outbreak.
Once again, the doctors and nurses raised their determination and tried their best to save the patient's life. After 3 months of treatment, with the continuous efforts of the doctors and the patient's family, the skin lesions recovered, the patient's health improved, and the patient was eligible for surgery to remove the tumor.
The surgery fortunately went smoothly despite the extremely difficult prognosis. Postoperatively, the patient was stable, and the skin and mucosal lesions recovered.
Today, after more than half a year, with the consensus of the medical staff of Bach Mai Hospital in general and the Dermatology Department in particular, patient Trinh has revived and has a new life, a new appearance, and a cheerful attitude.
Ms. Nguyen Thi Lan - the patient's mother, who has accompanied the patient for the past 6 months at Bach Mai Hospital, expressed her deep gratitude to the team of doctors and medical staff at Bach Mai Hospital. For Ms. Lan, over the past 6 months, the Hospital has become her second home and seems to be the place where her child was born again.
Source: https://baodautu.vn/dieu-tri-thanh-cong-cho-nguoi-mac-benh-hiem-tren-the-gioi-d218814.html
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