Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still’s disease: a case report and review of the literature

Michailidou, Despina; Shin, Ja Young; Forde, Inga; Gopalratnam, Kavitha; Cohen, Paul; DeGirolamo, Angela

doi:10.1007/s13317-015-0071-9

Cited by 10 publications

(4 citation statements)

References 38 publications

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“…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. The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis (especially those with persistent pruritic papules and plaques with dermatomyositis-type appearance), with a mortality rate that reached 8% primarily because of infectious complications related to the immunosuppressive therapy [6,10,24] .…”

Section: Discussionmentioning

confidence: 99%

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%

Adult-onset Still's disease with atypical cutaneous manifestations

García¹,

Pascual²,

Recalde³

et al. 2017

Medicine

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“…The mean age of these eight cases was 29.5 years (range, 23–47 years) (Table 1 ). [ 6 – 10 , 34 , 35 ] To the best of our knowledge, this patient represents the first reported case of severe SIRS complicating steroid-resistant AOSD that was successfully treated with anti-IL-6 antibody (Table 1 ).…”

Section: Discussionmentioning

confidence: 95%

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease

Masui-Ito

Okamoto²,

Ikejiri³

et al. 2017

Medicine

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Rationale:Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure.Patient concerns:A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight.Diagnoses:The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded.Interventions:We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration.Outcomes:The patient's systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody.Lessons:SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS.

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“…Atypical eruptions have been associated with a more severe disease course. 5 Other causes of flagellate dermatitis include bleomycin-induced drug eruptions, mechanical, zebra-like dermatomyositis, shiitake mushroom dermatitis, and jellyfish stings. 6 …”

Section: Discussionmentioning

confidence: 99%