Tổn thương da trong bệnh mô bào langerhans ở trẻ sơ sinh: nhân một trường hợp

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Tác giả: Thị Mai Hương Phạm, Thị Thùy Trang Trần

Ngôn ngữ: Vie

Ký hiệu phân loại:

Thông tin xuất bản: Tạp chí Y học Việt Nam 2023

Mô tả vật lý: 269-272

Bộ sưu tập: Metadata

ID: 410589

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Tóm tắt
Chủ đề

Bệnh mô bào Langerhans (Langerhans cell histiocytosis - LCH) là bệnh lý hiếm gặp do sự rối loạn phát triển tế bào đuôi gai. Tùy thuộc vào sự thâm nhiễm của tế bào đuôi gai ở các cơ quan trong cơ thể mà bệnh LCH có các biểu hiện lâm sàng khác nhau, trong đó da là một trong những cơ quan thường bị tổn thương nhất. Những biểu hiện trên da có thể gặp trong bệnh LCH như tổn thương giống viêm da dầu kèm theo các chấm xuất huyết, tổn thương dạng chàm, tổn thương dạng khối hoặc nốt và một số tổn thương hiếm gặp khác. Chúng tôi mô tả một trường hợp bệnh nhân sơ sinh với tổn thương da là các sẩn, nốt màu đỏ tím phân bố rải rác trên cơ thể. Trẻ được chẩn đoán mắc bệnh LCH sau khi có kết quả sinh thiết tổn thương. Ca lâm sàng: Trẻ nam 1 ngày tuổi, sinh thường đủ tháng, sau sinh trẻ hồng hào. Trẻ xuất hiện các tổn thương đa dạng trên da ngay sau sinh: sẩn, nốt màu đỏ tím rải rác ở đầu, mặt, lưng, bụng, lòng bàn tay xen kẽ dát màu hồng tím rải rác ở bụng, chân, tay. Trẻ được làm các xét nghiệm huyết học, TORCH, chẩn đoán hình ảnh và sinh thiết tổn thương da với kết quả giải phẫu bệnh: bệnh mô bào Langerhans. Qua tham khảo y văn cùng với biểu hiện lâm sàng và xét nghiệm, bệnh nhân được chẩn đoán mắc bệnh mô bào Hashimoto-Pritzker bẩm sinh tự giới hạn (Hashimoto-Pritzker congenital self-healing histiocytosis-HPH), một thể lâm sàng hiếm gặp của bệnh LCH. , Tóm tắt tiếng anh, Langerhans cell histiocytosis is a rare histiocytic disorder. Histiocytes accumulate in one or more organs, leading to a variable clinical presentation of disease, in which cutaneous manifestations is the most common. Cutaneous findings are found such as seborrheic dermatitis-like lesions with petechiae/hemorrhage lesions, eczematous lesions, papules/nodules/masses and several rare manifestations. We report a case of neonatal LCH with multiple red-brown cutaneous nodules and papules spread over his body. He was diagnosed LCH with biopsy-proven Langerhans Cell Histiocytosis. Clinical case: A full-term male neonate at 1 day of age, with normal birth weight and vaginal delivery, had multiple red-brown cutaneous nodules and papules on the head, face, trunk, palms and erythematous macules on the belly, extremities. He was done blood sample tests, TORCH, imaging studies with normal results and done biopsy with result: Langerhans Cell Histiocytosis. Combining of published medical research with cutaneous manifestations and proven biopsy, we diagnosed this neonate suffering from Hashimoto-Pritzker congenital self-healing histiocytosis, a rare congenital cutaneous LCH.

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