Adult-onset still's disease.

Elkon, Keith B.; Hughes, G. R. V.; Bywaters, E. G. L.; Ryan, P. F. J.; Inman, Robert D.; Bowley, N.; James, M.P.; Eady, R.A.J.

doi:10.1002/art.1780250607

Cited by 98 publications

(12 citation statements)

References 29 publications

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“…Histologically, AOSD patients have no specific inflammation of diagnostic significance3). Biopsies of skin rashes indicate only non-specific inflammatory reactions in the dermis, with mild or moderate perivascular infiltration by polymorphonuclear and/or mononuclear leukocytes1, 2). Histological analysis of mitral and aortic valve tissue of the present patient revealed chronic active inflammation with neutrophilic infiltration that was similar to previous skin histology.…”

Section: Discussionsupporting

confidence: 79%

Adult Onset Still's Disease as a Cause of Acute Severe Mitral and Aortic Regurgitation

Lee

Kang

et al. 2005

Korean J Intern Med

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Adult onset Still's disease (AOSD) is an uncommon acute systemic inflammatory disease of unknown origin. The clinical features include high spiking fever, arthralgia or arthritis, transient maculopapular rash, lymphadenopathy, hepatosplenomegaly, and serositis. Pericarditis is the most common cardiac manifestation of AOSD and occurs in approximately 30% of cases. A simultaneous occurrence of rapidly progressive bi-valvular regurgitation associated with AOSD has not been previously described. We report a case of a 55-year old woman who underwent mitral valve replacement and Bentall's operation due to acute severe mitral and aortic regurgitation associated with AOSD.

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Section: Discussionsupporting

confidence: 79%

Adult Onset Still's Disease as a Cause of Acute Severe Mitral and Aortic Regurgitation

Lee

Kang

et al. 2005

Korean J Intern Med

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“…Immunohistochemical stains may show complement and immunoglobulin deposits on the vessel walls. 10,12,13 Hence, the most characteristic feature of the rash remains its ephemeral nature.…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Still's disease with persistent plaques

Lübbe¹,

Hofer

Chavaz³

et al. 1999

British Journal of Dermatology

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Adult-onset Still's disease (AOSD) is a systemic disorder characterized by intermittent fever, evanescent rash, arthralgias or arthritis and predominantly neutrophilic leucocytosis. We report on a 16-year-old woman with Still's disease who developed, in addition to the typical rash, persistent papular lesions on her face, neck and upper and lower back. Although the presence of fixed skin lesions is not a characteristic feature of AOSD, their appearance at the onset of the disease and their evolution suggest that they represent a specific manifestation of the disease.

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“…The labs were unchanged from her AOSD baseline and the platelet and coagulation studies were within normal limits 2 3. The normal laboratory values and physical examination findings combined with the setting in which the skin findings occurred led to the diagnosis of RLP.…”

Section: Differential Diagnosismentioning

confidence: 95%

Rumpel-Leede phenomenon in a patient with adult-onset Still's disease

Lambdin¹,

Mackenzie

2017

BMJ Case Reports

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Rumpel-Leede phenomenon (RLP), also known as acute capillary rupture syndrome (ACRS), is a rare occurrence where distal dermal capillaries rupture in response to a proximal compressive force, such as a blood pressure cuff or tourniquet. This phenomenon has been reported to occur in states of vascular fragility such as long-term steroid use, hypertension or diabetes mellitus. Here, we provide a report of RLP occurring secondary to tourniquet application in a 26-year-old woman with adult-onset Still's disease (AOSD) and a recent drug rash. In this case, the cause of the phenomenon is most likely multifactorial. Likely contributing factors include long-term steroid use for the treatment of AOSD, and increased vascular permeability secondary to the drug rash. Patients and clinicians should be aware that the treatment of AOSD may induce a state of capillary fragility and they should work together to minimise the risk of complications.

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Adult-onset still's disease.

Cited by 98 publications

References 29 publications

Adult Onset Still's Disease as a Cause of Acute Severe Mitral and Aortic Regurgitation

Adult Onset Still's Disease as a Cause of Acute Severe Mitral and Aortic Regurgitation

Adult-onset Still's disease with persistent plaques

Rumpel-Leede phenomenon in a patient with adult-onset Still's disease

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