A Case of Dystrophic Epidermolysis Bullosa: Surgical Treatment for Hand Contracture Using Abdominal Skin Flap

Abstract

The patient was a 15-year-old male. Since birth, he has suffered from recurrent blistering, especially on the extremities. Histology and electron microscopic finding of the cutaneous lesions were compatible with those of dystrophic epidermolysis bullosa. Contracture and mobility limitation of the hands had gradually deteriorated. Prior to the admission to our clinic, both hands' contracture had been surgically treated 4 times in another hospital; however, after treatment with skin grafting, hand contracture recurred each time within one year. This time, surgical treatment was carried out for his left hand contracture using an abdominal skin flap under local anesthesia. In comparison with the free skin graft the abdominal skin flap improved hand contracture for a longer time. Abdominal skin flap is recommended as a method for the treatment of hand contracture of patients with dystrophic epidermolysis bullosa.

Free Anterolateral Thigh Perforator Flap for Reconstruction of Dystrophic Epidermolysis Bullosa-Associated Squamous Cell Carcinoma in the Foot: Case Report

“…3 Squamous cell carcinoma is a serious complication of epidermolysis bullosa and the prognosis is poor. 7,8,11 After a literature review, we discovered that there was only one case of recessive dystrophic epidermolysis bullosa with an associated malignancy in the hand that underwent successful reconstruction with a free scapular flap by Whitney. 2,6 Treatment of squamous cell carcinoma has been disappointing.…”

Epidermolysis bullosa dystrophica (Hallopeau-Siemens) an der Hand - Operative Strategie und Ergebnisse

“…Für die deformierte Hand bedeutet dies chirurgische Maßnahmen, gefolgt von intensiver Wundbehandlung, Schienenbehandlung und Physio-und Ergotherapie. An chirurgischen Verfahren wurden Lappenplastiken, wie zum Beispiel Bauchhautlappen [14], oder Lappenplastiken aus amputierten Fingern beschrieben [9].…”

Operative Behandlung deformierter Hände bei Epidermolysis bullosa dystrophica

“…In der Literatur reichen die Angaben von freien [21], Voll-und Spalthauttransplantaten [9,15,22] und gestielten Lappen [1,19] bis zu gezüchteten Hautzellkulturen [6] und Keratinozyten von kompatiblen Spendern [25]. In der Literatur reichen die Angaben von freien [21], Voll-und Spalthauttransplantaten [9,15,22] und gestielten Lappen [1,19] bis zu gezüchteten Hautzellkulturen [6] und Keratinozyten von kompatiblen Spendern [25].…”

“…Im Gegensatz zu vielen anderen Autoren wurde auf eine plastische Defektdeckung durch Hauttransplantate [1,15,19,22] oder gezüchtete Hautzellkulturen [6] verzichtet und die spontane Epithelialisierung abgewartet. Die Hauptpfeiler der Operation bestehen in der Dermolyse, Arthrolyse, Arteriolyse, Neurolyse und temporären Kirschner-Draht-Fixation.…”