Adult-onset Still’s disease: a report of 28 cases and review of the literature

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/microl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.

Urticaria and dermographism in patients with adult-onset Still’s disease

“…Interestingly, all three patients presented dermographism, a feature often observed in adult-onset StillЈs disease. Mehpoor et al [7], Chen et al [23] and Cagatay et al [24] found a dermographism incidence during disease activity of 92, 57 and 26.2%, respectively.…”

“…Therefore, a cutaneous rash in the setting of arthralgia and fever should raise suspicion of an adult-onset Still's disease, particularly if accompanied by dermographism [7]. This approach could provide early diagnosis and adequate treatment, thus preventing undesirable sequelae.…”

“…Cutaneous manifestations are common in this systemic disorder and include evanescent maculopapular rash with or without pruritus [1][2][3][4][5][6], dermographism [7] and Wxed erythematous papules/plaques [8][9][10][11][12][13]. However, only about 25 cases of AOSD are described in the literature which had a clinical presentation with urticaria or angioedema [8,[14][15][16][17][18][19][20][21][22].…”

Presentation and diagnosis of adult-onset Still’s disease: the implications of current and emerging markers in overcoming the diagnostic challenge

“…23 'Policyclic patter' b. 33 'Chronic articular' [121] 1200.94 ± 588.8 ng/ml 28 [122] 68-85,000 ng/ml 37 [123] 3540.26 ± 6701.9 ng/ml 39 [67] a. 13,000 ± 2200 ng/ml b.…”

Dermographism in a patient with fever

“…2 In some series, it has been observed even more frequently than the classic rash during active disease. 4 Lesions typically appear after scratching and fade in minutes or hours. On histopathology, an urticarial neutrophilic dermatosis pattern, with karyorrhexis but without vasculitis, has been described in these cases.…”