[Manifestations of scleroderma at the hand--options for hand surgery in an interdisciplinary concept].

Abstract

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.

• Authors: A Daigeler, M Meyer, H Joneidi-Jafari, C Möcklinghoff, H-U Steinau, M Lehnhardt
• Journal: Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V... (Handchir Mikrochir Plast Chir) Vol. 39 Issue 2 Pg. 128-34 (Apr 2007) ISSN: 0722-1819 [Print] Germany
• Vernacular Title: Handbeteiligungen im Rahmen der progressiven systemischen Sklerodermie--Möglichkeiten der Handchirurgie im interdisziplinären Konzept.
• PMID: 17497610 (Publication Type: Case Reports, English Abstract, Journal Article)

Topics

• Aged
• Amputation, Surgical
• CREST Syndrome (surgery, therapy)
• Calcinosis (surgery)
• Female
• Fingers (surgery)
• Follow-Up Studies
• Hand (surgery)
• Hand Deformities, Acquired (surgery)
• Humans
• Middle Aged
• Necrosis (surgery)
• Patient Care Team
• Raynaud Disease (surgery, therapy)
• Scleroderma, Systemic (complications, surgery, therapy)
• Sympathectomy
• Time Factors