Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Sun, Natalie; Brezinski, Elizabeth A.; Berliner, Jacqueline G.; Haemel, Anna; Connolly, M. Kari; Gensler, Lianne S.; McCalmont, Timothy H.; Shinkai, Kanade

doi:10.1016/j.jaad.2015.04.063

Cited by 45 publications

(42 citation statements)

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“…However, in recent years, other atypical cutaneous manifestations of AOSD have been reported; these rare presentations are not routinely recognized [6–38] Atypical cutaneous features often present in addition to the typical evanescent rash, but in 43% of the cases they are the only skin manifestation. [6,8,11,14,15,17,25,28–33,37,38] . A delayed diagnosis in these cases is still common, as the cutaneous lesions are often misdiagnosed as an allergic reaction to drugs, usually NSAIDs prescribed for joint symptoms or fever.…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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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“…Adult‐onset Still's disease (AOSD) is a systemic disease characterized by fever, arthralgia or arthritis, a cutaneous rash accompanied by elevation of inflammatory parameters and hyperferritinemia. The diagnosis rests upon the presence of a constellation of manifestations, and requires exclusion of other diseases, namely lupus erythematosus and rheumatoid arthritis (negative antinuclear antibodies and rheumatoid factor) …”

Section: Dyskeratotic Keratinocytes In the Hornmentioning

confidence: 99%

“…However, atypical persistent rashes have been increasingly recognized in the literature . These eruptions are typically composed of pruritic papules and plaques, with an erythematous, brown or rarely violaceous color.…”

Section: Dyskeratotic Keratinocytes In the Hornmentioning

confidence: 99%

‘Hints’ in the horn: diagnostic clues in the stratum corneum

et al. 2017

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Review'Hints' in the horn: diagnostic clues in the stratum corneumThe stratum corneum or horny layer is the uppermost layer of the epidermis, and is mainly responsible for the skin's barrier function. In spite of its complexity at the ultrastructural and molecular level, the features accessible to visualization on conventional histology are relatively limited. Nevertheless, knowledge of subtle clues that one may observe in the stratum corneum can prove useful in a wide range of situations in dermatopathology. We herein review a selection of common and rare entities in which the horny layer may reveal significantly important hints for the diagnosis. These clues include parakeratosis and its different patterns (focal, confluent, alternating, associated with spongiosis, epidermal hyperplasia or lichenoid changes), subcorneal acantholysis, infectious organisms in the stratum corneum (including fungal, bacterial and parasitic), thickening or thinning of the stratum corneum and the presence of different kinds of pigment. Even when normal, the horny layer may prove to be useful when seen in association with severe epidermal damage, a combination of features testifying to the acute nature of the underlying pathological process.Keywords: histopathology, hyperkeratosis, hypokeratosis, parakeratosis, stratum corneum Cardoso JC, Veraitch O, Gianotti R, Ferrara G, Tomasini CF, Singh M, Zalaudek I, Stefanato CM. 'Hints' in the horn: diagnostic clues in the stratum corneum.

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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%

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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Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy

Cited by 45 publications

References 50 publications

Adult-onset Still's disease with atypical cutaneous manifestations

Adult-onset Still's disease with atypical cutaneous manifestations

‘Hints’ in the horn: diagnostic clues in the stratum corneum

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

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