Acute adult-onset still’s disease presenting as pulmonary hemorrhage, urticaria, angioedema and leukemoid reaction: a case report and literature review

Sergio A Mora Alfonso, Daniel M Cuestas Rodríguez, John D Londoño, Rafael Valle-Oñate, Gerardo Quintana

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. 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. 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. 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.