Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study

Ding, Yu; Tang, Sha; Li, Sheng; Yang, Chunlin; Liu, Taoming; Ying, Shilong; Zhu, Dexi; Sun, Cheng; Chen, Weiqian; Shi, Yu; Fang, Hong; Qiao, Jianjun

doi:10.1016/j.jaad.2021.02.005

Cited by 9 publications

(9 citation statements)

References 27 publications

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“…10 In a recently published retrospective cohort study, researchers found that the presence of PPEs, AST > 120 U/L and splenomegaly were significantly correlated with MAS development in patients with SD. 2 However, the association between atypical persistent DM-like cutaneous eruption and MAS remains unknown.…”

Section: Reportmentioning

confidence: 99%

“…Although not included in the diagnostic criteria, persistent pruritic eruptions (PPEs) are frequent in SD, and they have distinctive clinical features and specific histological findings 1 . The presence of PPEs has been associated with worse prognosis and higher mortality rates 2–4 . We report two cases of severe SD with atypical persistent dermatomyositis‐like skin eruption, who developed secondary macrophage activation syndrome (MAS).…”

mentioning

confidence: 99%

“…1 The presence of PPEs has been associated with worse prognosis and higher mortality rates. [2][3][4] We report two cases of severe SD with atypical persistent dermatomyositis-like skin eruption, who developed secondary macrophage activation syndrome (MAS).…”

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confidence: 99%

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Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome

Camporro

Diaz

Beteta-Gorriti

et al. 2022

Clinical and Experimental Dermatology

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The typical rash of Still disease is an asymptomatic, salmon‐coloured, macular, or maculopapular eruption that appears along with the fever spikes and fades when the body temperature drops. Although not included in the diagnostic criteria, there are other frequent, persistent, pruritic and polymorphic skin manifestations of Still disease that have distinctive clinical features and specific histological findings. Among these atypical persistent pruritic eruptions (PPEs), periorbital erythema and linear flagellate papules and plaques can resemble the cutaneous manifestations of dermatomyositis (DM). The presence of these lesions in adult‐onset Still disease has been associated with worse prognosis and higher mortality rates, perhaps due to the development of a secondary macrophage activation syndrome (MAS). We report two cases of Still disease with persistent atypical DM‐like eruption, one in a paediatric patient (a very underreported finding) and the other in an adult. Both cases developed a secondary MAS.

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Section: Reportmentioning

confidence: 99%

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confidence: 99%

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Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome

Camporro

Diaz

Beteta-Gorriti

et al. 2022

Clinical and Experimental Dermatology

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“…Moreover, these atypical features may be associated with more severe symptoms and are theorized to be a negative prognostic factor for disease. [10][11][12] A cohort study including 150 patients with AOSD found that those with atypical persistent pruritic eruptions had higher levels of lactate dehydrogenase and ferritin and a higher rate of macrophage activation syndrome than patients with the typical evanescent rash. 10 Dyskeratotic cells on histology in persistent pruritic eruptions have been linked to an increase in IL-18 and subsequently a worse prognosis.…”

Section: Introduction Case Presentationmentioning

confidence: 99%

“…[10][11][12] A cohort study including 150 patients with AOSD found that those with atypical persistent pruritic eruptions had higher levels of lactate dehydrogenase and ferritin and a higher rate of macrophage activation syndrome than patients with the typical evanescent rash. 10 Dyskeratotic cells on histology in persistent pruritic eruptions have been linked to an increase in IL-18 and subsequently a worse prognosis. 11 Further, AOSD has been increasingly associated with delayed malignancies, commonly breast cancer and lymphoma.…”

Section: Introduction Case Presentationmentioning

confidence: 99%

Adult-Onset Still’s Disease with an Atypical Cutaneous Manifestation

et al. 2022

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Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder, characterized by daily fevers, arthralgias, and rash without evidence of infection. Recently there has been a growing number of cases reported with atypical cutaneous manifestations. We present a 37-year-old type VI Fitzpatrick skin type female who was seen in the hospital for a pruritic rash on her chest, back, and legs. Skin examination was significant for hyperpigmented papules coalescing into plaques on the chest, and hyperpigmented linear patches and excoriated plaques on the back and legs. Punch biopsy showed acanthosis and numerous apoptotic keratinocytes in the upper layers of the epidermis, which was characteristic of AOSD. As of recently, there is a growing body of evidence describing an atypical eruption of persistent, pruritic papules and plaques with typical histologic findings of AOSD. Further, these atypical features may be associated with more severe symptoms, higher incidence of macrophage activation syndrome, and possible delayed malignancy. Due to the scarcity of atypical cutaneous manifestations reported in the literature, these eruptions have not been included in the diagnostic criteria and can therefore be overlooked and misdiagnosed. Prompt recognition of these atypical eruptions is imperative to prevent a worsening prognosis and serious adverse effects.

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Association of atypical skin manifestations at the onset of systemic juvenile idiopathic arthritis with difficult-to-treat disease: A retrospective multicenter study

Eveillard¹,

Quartier²,

Ouldali³

et al. 2022

Journal of the American Academy of Dermatology

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Risk of macrophage activation syndrome in patients with adult-onset Still's disease with skin involvement: A retrospective cohort study

Cited by 9 publications

References 27 publications

Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome

Still disease with persistent atypical dermatomyositis-like skin eruption: two cases associated with macrophage activation syndrome

Adult-Onset Still’s Disease with an Atypical Cutaneous Manifestation

Association of atypical skin manifestations at the onset of systemic juvenile idiopathic arthritis with difficult-to-treat disease: A retrospective multicenter study

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