Urticaria and dermographism in patients with adult-onset Still’s disease

Criado, Paulo Ricardo; Carvalho, Jozélio Freire de; Ayabe, Liliane Akemi; Brandt, Hebert Roberto Clivati; Romiti, Ricardo; Maruta, Celina Wakisaka

doi:10.1007/s00296-011-2025-5

Cited by 23 publications

(27 citation statements)

References 20 publications

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“…In the great majority of cases, the atypical skin eruption presented at the time of disease onset concurrently with systemic symptoms, or shortly afterward. Rarely, cutaneous lesions were the presenting feature of AOSD preceding the appearance of the classic symptoms of the disease [9,13,23,29,30] or appeared during a flare several months after the diagnosis. [17,26,37] …”

Section: Discussionmentioning

confidence: 99%

“…The great majority of patients with AOSD and atypical cutaneous lesions had persistent and severe disease, with a considerable frequency (23%) of clinical complications, including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome [7,10,16–19,24,29,30,31,37] . Thus, most patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy consisting of immunosuppressant drugs (including methotrexate, azathioprine, cyclosporine A, and hydroxychloroquine) [6,8–10,12,15,16,18–20,24,25,28,29,31,34,39,40] and/or biologic agents (mainly anakinra or tocilizumab) [8,10,15,21] to control or manage symptoms because they had an intermittent/polycyclic or chronic systemic course.…”

Section: Discussionmentioning

confidence: 99%

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Adult-onset Still's disease with atypical cutaneous manifestations

García¹,

Pascual²,

Recalde³

et al. 2017

Medicine

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The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition.In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis.The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome.The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion.The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy with immunosuppressant drugs and/or biologic agents (mainly anakinra or tocilizumab) to control or manage symptoms because of a polycyclic or chronic course. The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis, with a mortality rate reaching 8% primarily because of infectious complications related to immunosuppressive therapy.In conclusion, the appearance of atypical cutaneous manifestations is not uncommon in AOSD. Recognition of this clinical variant is crucial for the early diagnosis of AOSD, as it might imply persistent disease activity and the need for more aggressive treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Adult-onset Still's disease with atypical cutaneous manifestations

García¹,

Pascual²,

Recalde³

et al. 2017

Medicine

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“…[6][7][8][9] Urticaria has also been reported. [3,10] Pruritic eruptions with linear lesions, as in one of our patients, have been reported as well, being described as dermographism by some authors [10] and a Koebner phenomenon by others. [11] Lee et al, have termed all atypical eruptions "persistent pruritic eruptions" and classified them clinically as urticarial papules, lichenoid papules, dermographism-like lesions, dermatomyositis-like lesions, prurigo pigmentosa-like lesions and lichen amyloidosus-like lesions.…”

Section: Discussion Discussionmentioning

confidence: 75%

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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“…Urticaria and urticaria-like eruptions have been reported in patients with AOSD [27][28][29][30]. Some of the reported cases may include typical rash of AOSD.…”

Section: Urticariamentioning

confidence: 95%

“…Some of the reported cases may include typical rash of AOSD. Patients with AOSD have a predilection for positive dermographism [30], suggesting that patients in active stage are susceptible to develop urticaria, like patients with rheumatoid arthritis and Sjögren's syndrome.…”

Section: Urticariamentioning

confidence: 99%

Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values

Yamamoto

2011

Rheumatol Int

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Adult-onset Still's disease (AOSD) is a systemic inflammatory condition, characterized by a high spiking fever, leukocytosis with neutrophilia, arthralgia, and skin rash. Typical skin rash is an evanescent, salmon-pink erythema predominantly involving extremities, which is included as one of the diagnostic criteria; however, recent findings show that not only typical evanescent rash but also various skin lesions are associated with AOSD. 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. Interestingly, persistent pruritic papules and plaques show unique histological features such as peculiar, distinctive distribution of dyskeratotic keratinocytes in the cornified layers as well as in the epidermis. Other non-classical skin lesions include urticaria. Current insights suggest that several inflammatory cytokines such as interleukin-1 (IL-1), IL-6, IL-18, interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) play a pathogenic role in AOSD. In particular, IL-18 is suggested to play a crucial role in activating macrophages, favoring Th1 type cytokine production. IL-18 induces IFN-γ, IL-17, and TNF-α, which may play an important pathogenic role in AOSD. It is important to recognize the common and/or uncommon skin conditions of AOSD for early correct diagnosis. In this review, various skin lesions are introduced, and the complication with histiocytic necrotizing lymphadenitis (Kikuchi disease) is further discussed.

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Urticaria and dermographism in patients with adult-onset Still’s disease

Cited by 23 publications

References 20 publications

Adult-onset Still's disease with atypical cutaneous manifestations

Adult-onset Still's disease with atypical cutaneous manifestations

Atypical cutaneous features in adult onset Still′s disease

Cutaneous manifestations associated with adult-onset Still’s disease: important diagnostic values

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