Adult-onset Still's disease with persistent plaques

Lübbe, Jann; Hofer, M; Chavaz, P.; Saurat, J.‐H.; Borradori, Luca

doi:10.1046/j.1365-2133.1999.03115.x

Cited by 55 publications

(54 citation statements)

References 10 publications

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“…This typical rash is usually asymptomatic and appears only with the fever. However, in recent years, cases with an atypical skin rash, severe pruritus and persistent plaques have been increasingly reported [1, 2, 3]. We report here a patient with an atypical skin rash, characterized by persistent papules and plaques causing linear pigmentation.…”

Section: Introductionmentioning

confidence: 89%

Persistent Plaques and Linear Pigmentation in Adult-Onset Still’s Disease

et al. 2001

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A 25-year-old Japanese man presented with high spiking fever, arthralgia and a skin rash. A pruritic edematous erythema with persistent plaques was found mainly on the trunk; these lesions persisted even when the fever subsided, with prominent linear pigmentation. As marked neutrophilia and a high level of serum ferritin were detected, a diagnosis of adult-onset Still’s disease (AOSD) was made, even though the persistent eruption was not characteristic of the disease. Oral prednisolone, together with low-dose methotrexate, was given with good results. In the literature, a similar atypical rash has been reported in 11 cases in Japan. All of them required high-dose administration of corticosteroids or other immunosuppressive agents. Severe systemic complications were seen in 3 patients, and 2 cases died of the disease. Persistent plaques and linear pigmentation are some of the manifestations of AOSD, which cannot be overlooked. This appearance could be an indication that suggests an increased risk of systemic complications and a prolonged time to clinical remission.

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Section: Introductionmentioning

confidence: 89%

Persistent Plaques and Linear Pigmentation in Adult-Onset Still’s Disease

et al. 2001

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“…As Still's rash is evanescent, a fixed rash is thought to be atypical in AOSD, and has been reported rarely as acne or eczematoid lesions (9), purpura with histological vasculitis (4), urticarial lesions (10), and peau d'orange-like infiltrated lesions (13). Previous reports have described erythematous confluent papules and plaques in AOSD, but these resolved synchronously with other systemic symptoms upon treatment (14,15). Our patient presented with a 2-month history of vesiculopustules limited to both hands and feet rather than typical Still's rash, which were difficult to observe due to the natural pigmentation.…”

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confidence: 83%

Adult-onset Still's Disease with Vesiculopustules on the Hands and Feet

et al. 2002

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Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology characterized by an evanescent rash, intermittent spiking high fever, arthralgia, and a variety of systemic features. We describe a 46-yr-old woman with Still s disease who presented with a 2-month history of a symmetrical vesiculopustular eruptions only on both hands and feet. Skin biopsy specimens of both vesicle and pustule revealed fibrin thrombi deposition in the small dermal vessels with little inflammation, subepidermal bulla, and ischemic necrosis of the overlying epidermis, which were consistent with a vasculopathic reaction. Her skin lesions gradually disappeared in response to conventional AOSD therapy, as elevated serum ferritin levels, an index of disease activity, decreased. To the best of our knowledge, this is the first case of vesiculopustular lesions as a skin manifestation of AOSD.

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“…Such skin lesions had been considered atypical for AOSD. However, recent findings show that not only typical evanescent rash but also various skin lesions are associated in AOSD [11][12][13][14][15] . The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques which presents erythematous, slightly scaly papules with linear configuration on the trunk and proximal extremities [12] .…”

Section: Discussionmentioning

confidence: 99%

Adult-onset Still’s disease in an elderly woman with atypical presentation

Kumano

Ishibashi

Kubota

et al. 2014

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We report an elderly case of adult-onset Still's disease (AOSD) with atypical presentation. The patient, an 80-year-old female, visited our hospital due to itchy linear urtical rash with low grade fever which occurred after taking a significant amount of blowfish milt four days before. Oral prednisolone of 30mg/day was successful. However, remittent fever appeared after reducing the prednisolone dose accompanied by the similar skin lesion. Hyperferritinemia was evident on the admission and the diagnosis of AOSD was made. We should keep in mind the presence of AOSD with atypical presentation in elderly patients.

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Adult-onset Still's disease with persistent plaques

Cited by 55 publications

References 10 publications

Persistent Plaques and Linear Pigmentation in Adult-Onset Still’s Disease

Persistent Plaques and Linear Pigmentation in Adult-Onset Still’s Disease

Adult-onset Still's Disease with Vesiculopustules on the Hands and Feet

Adult-onset Still’s disease in an elderly woman with atypical presentation

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