Daigeler A, Meyer M, Joneidi-Jafari H, Möcklinghoff C, Steinau H-U, Lehnhardt M
Universitätsklinik für Plastische Chirurgie und Schwerbrandverletzte, Handchirurgiezentrum, Operatives Referenzzentrum für Gliedmassentumoren, Berufsgenossenschaftliche Kliniken Bergmannsheil, Bürkle-de-la-Camp Platz 1, 44789 Bochum.
Handchir Mikrochir Plast Chir. 2007 Apr;39(2):128-34. doi: 10.1055/s-2006-924536.
Clinical manifestations of scleroderma at the hand include Raynaud's phenomenon, calcinosis cutis, sclerodactylia and teleangiectasia. With the progression of the disease, cutaneous and joint contractions, acro-osteolysis, necrosis of the finger tips, and even extensive digital ulceration are likely to occur. These painful and often rapidly advancing lesions cause loss of function and disfigurement and, untreated, often lead to mutilation of the affected hand. Only an interdisciplinary management including the hand surgeon, the rheumatologist, and the physiotherapist can guarantee optimal treatment. Drug therapy should be included as well as physical therapy. Both should be made use of before and accompanying surgical treatment. Surgical therapy consists of treatment of the infections, excision of calcinosis, arthrodesis, in particular of the proximal interphalangeal joints, and sympathectomy. Amputation remains a final option, whereas with timely and sufficient treatment, amputations can be avoided and an improvement of function and an alleviation of the symptoms can be achieved. Among the non-operative treatment options, behavioural training, calcium antagonists, prostacyclin derivatives, topical nitrates as well as plexus anesthesia and stellatum blocks have proved to be effective. Recent drug therapies include endothelin-receptor antagonists for the prevention of digital ulceration and phosphodiesterase-V antagonists in treatment of Raynaud's phenomenon and induction of ulcer healing. With reference to several cases seen at our institution, we propose an interdisciplinary treatment concept for acral manifestations of scleroderma.
手部硬皮病的临床表现包括雷诺现象、皮肤钙质沉着、指(趾)硬皮病和毛细血管扩张。随着疾病进展,皮肤和关节挛缩、肢端骨质溶解、指尖坏死,甚至广泛的指(趾)溃疡都可能发生。这些疼痛且往往迅速进展的病变会导致功能丧失和毁容,若不治疗,常导致患手残缺。只有包括手外科医生、风湿病学家和物理治疗师在内的多学科管理才能保证最佳治疗效果。药物治疗和物理治疗都应包括在内。两者都应在手术治疗前及手术治疗过程中使用。手术治疗包括感染治疗、钙质沉着切除术、关节固定术,尤其是近端指间关节的关节固定术,以及交感神经切除术。截肢仍是最后的选择,而通过及时、充分的治疗,可以避免截肢,并实现功能改善和症状缓解。在非手术治疗方法中,行为训练、钙拮抗剂、前列环素衍生物、局部硝酸盐以及神经丛麻醉和星状神经节阻滞已被证明是有效的。最近的药物治疗包括用于预防指(趾)溃疡的内皮素受体拮抗剂,以及用于治疗雷诺现象和促进溃疡愈合的磷酸二酯酶 -5 拮抗剂。参照我们机构所见的几例病例,我们提出了一种针对硬皮病肢端表现的多学科治疗理念。