title: Acute adult-onset still's disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review.
abstract: BACKGROUND
  Adult-onset Still's disease is a rare systemic inflammatory disorder of unknown aetiology characterized by the classic triad of persistent high spiking fevers, joint pain and a distinctive salmon-colored bumpy rash however, the multiorgan involvement can be present.
  
  
  METHODS
  A 40-year-old woman previously healthy was referred to our hospital with 7 days of high fever and generalized arthralgia, The physical exam revealed angioneurotic edema detected on soles, palms and tongue and widespread red, urticated plaques in a symmetrical distribution affecting the arms, dorsal hands, upper and lower chest and back. Followed 5 days later by fever, the patient presented dyspnea, cough and hypoxemia, the imaging studies showed unilateral consolidation and pleural effusion. The bronchoscopy with bronchoalveolar lavage and skin biopsy were consistent with neutrophilic urticarial. The hematological disorders, infections and other autoimmune diseases were excluded.
  
  
  RESULTS
  The diagnosis of adult-onset Still's disease can be very difficult. There are no specific tests and reliance is usually placed on a symptom complex and the well described typical rash seen in most patients. In recent years, however, other cutaneous manifestations of Adult-onset Still's disease have been reported but these are not so well known.
  
  
  CONCLUSIONS
  The evidence of rare manifestations is growing and the early clinical presentation of Adult-onset Still's is extremely variable, making diagnosis difficult. For this reason, data on early clinical presentation of the disease are of interest. We reported the first case of acute Adult-onset Still's disease with the association of pulmonary hemorrhage, urticaria and angioedema including a rare systemic manifestation as leukemoid reaction.
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, Hospital Universitario De La Samaritana E.S.E, Bogotá, Colombia.
authors.forename: Sergio A
authors.initials: SA
authors.lastname: Mora Alfonso
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, Hospital Universitario De La Samaritana E.S.E, Bogotá, Colombia ; Rheumatology Unit, Department of Internal Medicine, Universidad de La Sabana, Hospital Universitario de La Samaritana E.S.E, Bogotá, Colombia ; Clinical Rhematology Research Fellow, Rheumatology Unit, Hospital Universitario De La Samaritana E.S.E, Cra 18A # 10 - 25 sur, Bogotá, Colombia.
authors.forename: Daniel M Cuestas
authors.initials: DM
authors.lastname: Rodríguez
authors.affiliation: Department of Rheumatology, Universidad de La Sabana, Bogotá, Colombia.
authors.forename: John D
authors.initials: JD
authors.lastname: Londoño
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Hospital Militar Central, Bogotá, Colombia.
authors.forename: Rafael
authors.initials: R
authors.lastname: Valle-Oñate
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Universidad Nacional de Colombia, Hospital Universitario Fundación Santa Fe, Bogotá, Colombia.
authors.forename: Gerardo
authors.initials: G
authors.lastname: Quintana
keywords: Adult-onset still’s disease; Angioedema; Leukemoid reaction; Pulmonary hemorrhage; Urticaria