Kim Young Seok, Hong Hyun Joon, Roh Tai Suk
Department of Plastic & Reconstructive Surgery, Institute for Human Tissue Restoration, Yongdong Severance Hospital, Yonsei University College of Medicine, 146-92 Dogok-Dong Gangnam-Gu, Seoul, Korea.
J Plast Reconstr Aesthet Surg. 2009 Jul;62(7):e191-3. doi: 10.1016/j.bjps.2009.03.012. Epub 2009 Apr 25.
Pseudoainhum is a rare condition of unknown aetiology that is characterised by the presence of constriction bands of extremities due to a variety of underlying causes that progress to irreversible damage and auto-amputation of the affected digit. Epidermolysis bullosa is a group of disorders characterised by extremely fragile skin and recurrent blister formation resulting from minor mechanical trauma. The association of epidermolysis bullosa with other hand defects, such as hand contracture and pseudosyndactyly, have been reported; however, ainhum-like digital constriction bands have not been reported in association with this disorder. To our knowledge, this is the first reported case of pseudoainhum in association with epidermolysis bullosa and highlights good surgical response obtained with microscopic removal of the recurred constriction band and cross-finger flap to prevent recurrence, relieve symptoms and improve cosmesis.
自发性断指症是一种病因不明的罕见病症,其特征是由于多种潜在原因导致四肢出现缩窄带,进而发展为受影响手指的不可逆损伤和自行截断。大疱性表皮松解症是一组以皮肤极其脆弱以及因轻微机械创伤反复形成水疱为特征的疾病。大疱性表皮松解症与其他手部缺陷,如手部挛缩和假性并指的关联已有报道;然而,尚未有与该病症相关的类自发性断指症缩窄带的报道。据我们所知,这是首例关于大疱性表皮松解症合并自发性断指症的报道,突出了通过显微镜下切除复发的缩窄带和采用交叉手指皮瓣手术获得的良好效果,可防止复发、缓解症状并改善外观。