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

Abstract

Introduction: 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.

Case description: 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.

Discussion and evaluation: 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.

Keywords: Adult-onset still’s disease; Angioedema; Leukemoid reaction; Pulmonary hemorrhage; Urticaria.

Figure 1

Unusual clinical findings of AOSD in skin and hands. A photography of dorsal and palms aspect of hands shows marked angioedema (a). The urticarial eruption is evident in the abdominal photography and shows the red and urticated plaques (black arrows) (b).

Figure 2

Unusual Paraclinical findings of AOSD in lung. A chest radiograph shows a patchy right lobe consolidation in the lower lung zone (a). CT image shows extensive areas of airspace consolidation due to alveolar hemorrhage in right lung. The bilateral pleural effusions are also notable (b).

Figure 3

Abdominal skin biopsy with haematoxylin-eosin-stained. Perivascular and interstitial infiltrate composed mainly of neutrophils on upper and middle dermis with original magnification x 20 (a). Shows the same specimen in more detail with original magnification x 100 (b).