The Department of Plastic Surgery and Interdisciplinary Surgery, Central Tropical Diseases Hospital has just received and treated patient V.T. M, 54 years old, Hai Phong. The patient was diagnosed with Hailey-Hailey disease - a rare hereditary dermatology disease with a incidence rate of about 1/50,000 people.
When admitted to the hospital, the patient showed many areas of red, swollen, squamous and ulcerated skin and widespread discharge in both sides of the neck, armpits, breast folds, groin, elbows and ankles. The lesions caused prolonged pain, seriously affecting daily life and quality of life.
Sharing with the doctor, the patient said that she had been ill for 6 years, of which the situation has become increasingly serious in the last 2 years. Consecutive outbreaks caused the skin area at the folds to always be in a state of ulcers and burning pain. Although treatment has been provided at many medical facilities in Hanoi and Hai Phong, the lesions only subsided for a short time and then quickly recurred.
Notably, family history exploitation shows that the patient's father, uncle and younger sister also had a similar condition, showing a clear genetic factor of this disease.
According to Dr. Pham Minh Tam, dermatologist, Department of Plastic Surgery and Interdisciplinary Surgery, Hailey-Hailey disease is a disease caused by a gene mutation that disrupts the connection between epidermal cells. The consequence is that the skin becomes loose, easily cracks and forms blisters when there is friction or in hot and humid conditions, increasing sweat secretion.
This is not a simple infection or common atopic dermatitis, but a chronic disease with hereditary properties. High temperatures, increased sweating, friction, or bacterial, viral, and fungal superinfection can all cause the disease to break out more violently," said Dr. Tam.
After examination and necessary tests, doctors developed a medical treatment regimen combining oral and topical medications and controlling the superinfection. To reduce the risk of long-term recurrence, patients were prescribed Botulinum toxin A (Botox) injections to reduce sweating in the folded areas.
According to doctors, reducing sweating helps limit the humid environment - a favorable factor for disease recurrence. Due to the large area of damage, excessive discharge and patient pain, the treatment team cannot perform local anesthesia as usual but must perform procedures in the operating room with pain relief support to ensure safety.
After 7 days of treatment, the ulcerated skin areas of the patient were dry, no more discharge, and the pain was significantly reduced. The patient was discharged from the hospital and continued to have monthly periodic check-ups for monitoring and maintenance treatment.
According to Dr. Pham Minh Tam, Hailey-Hailey is a rare hereditary skin disease. Although there is currently no cure, if diagnosed correctly and the triggering factors are well controlled, patients can still limit recurrence and significantly improve their quality of life.
