Surgical Correction of Syndactyl Deformities of Hand and Feet in Epidermolysis Bullosa-A Case Report

大疱性表皮鬆解症手腳併趾畸形的外科矯正－一病例報告

賴志昇(Chih-Sheng Lai) ； 陳伊呈(I-Chen Chen) ； 唐友文(Yu-Wen Tang)

《中華民國整形外科醫學會雜誌》 18卷4期 (2009/12) Pp. 362-372

https://doi.org/10.7096/JPSA.200912.0362

摘要

背景：
大疱性表皮鬆解症是一種罕見疾病，通常有遺傳性、生命的早期發病、導致受傷部位有大水疱與糜爛腐蝕形成，通常發生在手（遍及關節）、手肘、膝蓋和腳。
目的及目標：
手術治療的主要目標是拯救生命和藉由外科重建改善肢體功能的畸形與攣縮。
材料及方法：
我們報告一位兩個月大的女孩，從一出生就有手掌、足掌皮膚糜爛腐蝕情形。皮膚的損害進展成許多的大水疱與手掌、足掌未發炎的糜爛腐蝕，導致手腳的併指畸形與屈曲攣縮。
結果：
在連續外科手術之後，病患恢復有用的手部功能與令人滿意的腳外觀，包括趾間蹼的放鬆、疤痕攣縮的放鬆、局部皮瓣的移位與全層植皮。我們報告關於大疱性表皮鬆解症的手和腳外科重建的經驗。

並列摘要

Background:
Epidermolysis bullosa (EB) is a rare disease that is usually inherited, begins early in life, and results in bullae and erosion at sites of trauma, usually on the hands (over joints), elbows, knees, and feet.
Aim and Objectives:
The primary goal of surgical treatment is to save life and improve the function of the involved extremities through surgical reconstruction of deformities and contractures.
Materials and Methods:
We report a case of a 2-month-old girl with eroded skin over both hands and feet since birth. The skin lesions progressed with numerous grouped bullae, and erosions on a noninflamed base on the palms and soles which resulted in syndactyl deformities and flexion contracture of hands and feet.
Results:
After serial surgical procedures including web release, contracture release, local flap transposition and full thickness skin graft, the patient regained useful hand function and an acceptable appearance of both feet. We report our experience of surgical reconstruction of hands and feet due to EB.

並列關鍵字

epidermolysis bullosa ； syndactyl deformities