Adult‐onset Still's disease with atypical cutaneous features

Affleck, AG; Littlewood, S.M.

doi:10.1111/j.1468-3083.2004.01142.x

Cited by 41 publications

(32 citation statements)

References 16 publications

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“…7,[10][11][12][13][14][15][16][17]20,21 In these latter cases, atypical skin rash appeared as soon as 1 week 18 and as late as 5 months 10 after the classic symptoms of Still disease. The duration of the atypical eruption before presentation for evaluation ranged from 2 to 3 weeks 14,16,20 to 1 year.…”

Section: Literature Review Of Clinical Presentations In Patients Withmentioning

confidence: 99%

“…2 Recently, small studies and case reports have described an atypical, persistent skin rash consisting of pruritic papules and plaques with various configurations with distinct histopathology in patients with active AOSD. [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] This atypical rash often presents in addition to the typical rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In addition to these reports of atypical cutaneous presentations, emerging literature highlights an increasing recognition of malignancy in association with Still disease, sometimes occurring years later.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Sun

Brezinski

Berliner

et al. 2015

Journal of the American Academy of Dermatology

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Section: Literature Review Of Clinical Presentations In Patients Withmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Sun

Brezinski

Berliner

et al. 2015

Journal of the American Academy of Dermatology

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“…In some studies, dermatographism and Köebner phenomenon have been described as objective features of AOSD [5,6,9,19]. Various atypical rashes have been reported in AOSD such as persistent dermal plaques, [20][21][22][23] urticaria [19,24], and vesicolupustular lesions on the hands and feet [25].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2008

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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.

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“…Therefore, the frequent delay on the diagnosis brings no surprises and our case is no exception. The lack of specificity of the symptoms and signs, and their absence of synchronicity concerning the cutaneous manifestations, represent a strong contributor to the difficulties on this diagnosis [9][10][11].…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Still’s Disease in the Adult

et al. 2017

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Adult Still's disease (ASD) is a rare systemic inflammatory disorder of unknown etiology, typically characterized by a clinical triad (daily spiking high fevers, evanescent rash, and arthritis) and a biological triad (hyperferritinemia, hyperleucocytosis with neutrophilia and abnormal liver function test). There are no specific diagnostic tests for ASD, so the diagnosis of ASD remains one of exclusion and the differential diagnosis may be lengthy. We present a case of an adultonset Still's disease with previous admissions for fever of unknown origin with a 2-month history of fever and systemic symptoms. The patient posteriorly presented polyarthralgies and cutaneous rash. The study disclosed anemia, inflammatory markers and hepatic enzymes elevation, and negative serological and immunological studies. The introduction of corticotherapy resolved symptoms and laboratories alterations. Adult-onset Still disease is a heterogeneous and rare disease and the lack of serologic markers as a true gold standard makes diagnosis difficult.

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Adult‐onset Still's disease with atypical cutaneous features

Abstract: The diagnosis of AOSD can be made in the absence of the typical Still's rash but in the presence of other atypical cutaneous features.

Cited by 41 publications

References 16 publications

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

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

A Case Report of Still’s Disease in the Adult

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