Diagnosis of rare diseases from skin lesions

Hà Lê |

A male patient over 30 years old was hospitalized with many bruises, painful swollen spots and skin necrosis. Through examination, doctors determined that he had EGPA - an extremely rare vascular disease.

The patient was admitted to the hospital in a state of appearing many subcutaneous nodules swollen, red, painful, accompanied by purple necrotic rashes scattered in the hands, feet and body, concentrated in both lower limbs. The lesions caused a lot of pain, significantly affecting daily life and quality of life.

After clinical examination, doctors prescribed a skin biopsy to perform anatomy tests for the disease. Tissue pathology results record images of small and medium-sized vascular inflammation with the advantage of eosinophilic polycytes, accompanied by granulomatous reactions and fat tissue inflammation. These are typical signs, strongly suggesting EGPA diagnosis.

After professional consultation, the patient was diagnosed with granulomatous leukemia with polycystic acidosis accompanied by polyvascular inflammation. At the same time, doctors conducted a comprehensive assessment to detect lesions in common internal organs of the disease such as lungs, kidneys and other organs to develop appropriate treatment regimens.

After a week of treatment, the inflammation spots decreased significantly, the swelling and pain situation almost completely disappeared, and the ulcerated lesions tended to heal well. The patient was discharged from the hospital in stable condition and continued to be monitored periodically at the Vascular Inflammatory Clinic to control the disease for a long time.

According to Dr. Vu Huy Luong, Head of the Specialist Group on Vascular Inflammatory Diseases, Head of Laser and Skin Care Department, Central Dermatology Hospital, EGPA - a small and medium-sized vascular disease that is very rare, especially in Asians - is an extremely rare vascular disease in the world as well as in Asia. Statistics in the US and Europe show that the incidence rate is only about 0.5-3 cases per million people per year, while in Asia, only individual cases have been recorded.

This is an autoimmune disease that causes inflammation of small and medium-sized blood vessels, characterized by the invasion of polycytes and the formation of granulomatous lesions. The disease can manifest as many skin symptoms such as hemorrhagic rash, subcutaneous spots, ulcers, skin necrosis or purple patches, and also cause damage to many internal organs, especially the lungs, peripheral nerves, heart and kidneys.

If not detected and treated promptly, EGPA can progress severely, causing multiple organ failure, even life-threatening. Therefore, early detection of skin signs is very important, helping to diagnose early and prevent serious complications," Dr. Vu Huy Luong emphasized.

According to experts, in the acute inflammation phase, patients are often treated with high-dose immunosuppressants, then gradually reduced according to clinical response and monitored for a long time to control the disease.

Hà Lê
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