title: [Digital ulcers in systemic sclerosis: use of endotheline antagonists].
abstract: BACKGROUND
  Systemic sclerosis (SSc) is a systemic disease, characterized by fibrosis and vasculopathy, with variable internal organ involvement. Skin is very often involved, namely digital ulcers (DU), seldom treatment resistant, responsible for important functional limitation. The DU can evolve from sclerodactily with superficial ulcers, isquemic lesions, deep necrosis, gangrene, loss of tissue, and consequently, to finger amputation.
  
  
  METHODS
  The authors describe the case of a 36 year old female patient, with SSc diagnosed 6 years previously, with skin, lung and gut manifestations. The patient showed uncontrolled Raynaud's phenomenon (RF), despite the adequate treatment using nifedidpine and general local warming measures, with progressively worsening DU and isquemia, especially in cold seasons. Bosentan, 62.5 mg twice daily was started, and a significant improvement in the peripheral isquemic lesions was achieved. The ulcers' healing was fast, the patient totally recovered function and regained quality of life, and no further lesions developed.
  
  
  CONCLUSIONS
  The authors review the RF and DU in SSc, as well as the use of bosentan, an endotheline receptor antagonist, and its indications. Although it is not formally approved, the use of bosentan in SS has shown benefits in reducing the incidence of DU, and despite no influence in the healing process, this drug prevents the development of new lesions.
authors.affiliation: Serviço de Medicina Interna, Hospitais da Universidade de Coimbra, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Mota
authors.forename: Amparo
authors.initials: A
authors.lastname: Castellano
authors.forename: Felicidade
authors.initials: F
authors.lastname: Santiago
authors.forename: Patrícia
authors.initials: P
authors.lastname: Carvalho
authors.forename: A Sofia
authors.initials: AS
authors.lastname: Madeira
authors.forename: J M
authors.initials: JM
authors.lastname: Pereira De Moura
authors.forename: J M
authors.initials: JM
authors.lastname: Nascimento Costa
mesh_terms: D000328:Adult; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D005260:Female; D005385:Fingers; D006801:Humans; D012595:Scleroderma, Systemic / Q000150:complications*; D012883:Skin Ulcer / Q000188:drug therapy* / Q000209:etiology*; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan