Adult‐onset Still’s disease with dermatomyositis‐like eruption

Ohashi, Masafumi; Moriya, Chie; Kanoh, Hiroyuki; Kinomura, Yujiro; Terakura, Yoichi; Kasahara, Senji; Hamaguchi, Yasuhito; Fujimoto, Manabu; Seishima, Mariko

doi:10.1111/j.1346-8138.2012.01549.x

Cited by 11 publications

(13 citation statements)

References 4 publications

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“…Clinical features of this presentation are varied, but the most commonly described features are erythematous, brown or violaceous papules and plaques that frequently take on a linear configuration, which may be a reflection of Koebner phenomenon . The rash is usually found on the chest and back, and in some cases has been described as photodistributed simulating autoimmune connective diseases such as lupus erythematosus and dermatomyositis . The persistent rash of AOSD appears to affect young Asian women more frequently, at least based on published reports, corroborated by patients herein.…”

Section: Discussionmentioning

confidence: 55%

Clinical and histopathological features of cutaneous manifestations of adult‐onset Still disease

et al. 2017

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Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils. Later reports described a more persistent rash that tended to be photodistributed, hyperpigmented, often in a linear configuration, sometimes in a rippled pattern, referred to as the atypical eruption of AOSD. The presence of individual necrotic keratinocytes in the upper spinous layer has been the consistent histopathologic finding. The persistent rash may not represent an atypical presentation of AOSD as recent reports indicate a high prevalence of the rash. Emerging data also suggest that patients with persistent eruption have a worse prognosis. The recognition of the clinical and histopathological findings of skin eruptions of AOSD may facilitate an earlier diagnosis, potentially improving disease outcome. Herein, clinical and histopathological features of cutaneous manifestation of AOSD in 2 Asian women are highlighted accompanied by a relevant review of the disease.

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Section: Discussionmentioning

confidence: 55%

Clinical and histopathological features of cutaneous manifestations of adult‐onset Still disease

et al. 2017

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“…[7,12,13] Although the typical rash has a relatively high sensitivity and specificity, some forms of atypical rashes may have prognostic significance. For instance, persistent plaques, linear pigmentation and dermatomyositis-like eruptions have been associated with a more severe clinical course [12,14] and suggested to be a clinical marker of disease severity. [6,9] Such cutaneous manifestations may therefore be an indication for the institution of more aggresive systemic treatments.…”

Section: Discussion Discussionmentioning

confidence: 99%

Atypical cutaneous features in adult onset Still′s disease

Akkurt

Bozkurt

Uçmak

et al. 2014

Indian J Dermatol Venereol Leprol

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Adult onset Still's disease is a rare but potentially serious disease. We present five cases of adult-onset Still's disease seen by us over a period of one year. The patients were all females and 28-39 years of age. Symptoms had been present for 2-6 weeks in three patients. The other two had been on a few years' follow-up for rheumatoid arthritis before the onset of rashes and fever. The patients had persistent erythematous maculopapular eruptions on face, body and extremities, with moderate to severe pruritus and/or a burning sensation that decreased their quality of life. The typical evanescent rash was not observed. High ferritin values were detected in all the patients and total serum IgE was increased in two. All the patients were started on oral prednisolone (0.5-1.0 mg/kg/day), and methotrexate (10-15 mg/week) had to be added in three patients. One patient was started on tocilizumab due to recalcitrant disease and one was lost to follow-up. Further investigation and classification of the various atypical cutaneous findings in adult-onset Still's disease is necessary.

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“…These lesions usually present as papules and plaques on the neck, back, and face . Several case series and case reports have described the clinical and histological features of PPEs . Dyskeratosis is found mainly in the upper epidermis and cornified layers, whereas lymphocyte and neutrophil infiltration occurs in the superficial dermis .…”

Section: Introductionmentioning

confidence: 99%

“…6,7 Several case series and case reports have described the clinical and histological features of PPEs. 4,[7][8][9][10][11][12][13][14][15] Dyskeratosis is found mainly in the upper epidermis and cornified layers, whereas lymphocyte and neutrophil infiltration occurs in the superficial dermis. [8][9][10]12,13 Neutrophil exocytosis, basal vacuolar alterations and infiltration of eosinophils have also been reported in some cases.…”

Section: Introductionmentioning

confidence: 99%

Histopathological diagnosis of persistent pruritic eruptions associated with adult‐onset Still's disease

Qiao

Zhou

et al. 2019

Histopathology

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Aims Persistent pruritic eruptions (PPEs), presenting with dyskeratotic keratinocytes histologically, are characteristic skin rash in patients with adult‐onset Still's disease (AOSD). The lesions may be histologically similar to other entities that present with dyskeratosis. In the present study, we compared the histopathological features between PPEs and other entities presenting with dyskeratosis. Methods and results To investigate whether histopathological findings can be used to discriminate among PPEs and other entities presenting with dyskeratotic keratinocytes, cutaneous histopathological changes of PPEs associated with AOSD (n = 26) were compared with those of systemic lupus erythematosus (SLE) (n = 16), dermatomyositis (n = 19), and drug eruption (n = 16). Dyskeratosis was observed in the upper one‐third of the epidermal layer in all 26 PPEs. The rate of dyskeratosis for PPEs was higher than that for SLE (18.8%) and dermatomyositis (15.8%). In drug eruptions, the dyskeratotic cells were distributed in all levels of the epidermis. Variable densities of neutrophils were found in the dermis in all PPEs. Conclusions Although this was a retrospective study conducted at a single centre, presentation of dyskeratotic keratinocytes in the upper one‐third of the epidermal layer is a distinctive histopathological reactive pattern of PPEs. This pattern may be a useful histopathological marker for early diagnosis of AOSD.

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Adult‐onset Still’s disease with dermatomyositis‐like eruption

Cited by 11 publications

References 4 publications

Clinical and histopathological features of cutaneous manifestations of adult‐onset Still disease

Clinical and histopathological features of cutaneous manifestations of adult‐onset Still disease

Atypical cutaneous features in adult onset Still′s disease

Histopathological diagnosis of persistent pruritic eruptions associated with adult‐onset Still's disease

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