A comprehensive review on adult onset Still's disease

Giacomelli, Roberto; Ruscitti, Piero; Shoenfeld, Yehuda

doi:10.1016/j.jaut.2018.07.018

Cited by 311 publications

(340 citation statements)

References 185 publications

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“…The diagnosis of AOSD is based on excluding other conditions, including autoimmune diseases, infectious diseases, and malignancies. The diagnosis is challenging and often delayed, as no specific markers are available in the clinic and laboratory …”

Section: Introductionmentioning

confidence: 99%

“…The rash disappears when the fever subsides . Histologically, evanescent rash shows superficial perivascular inflammation with sparse neutrophil infiltration . Evanescent rash has traditionally been a major and important diagnostic criterion for AOSD …”

Section: Introductionmentioning

confidence: 99%

“…Adult-onset Still's disease (AOSD) is an uncommon autoinflammatory disease that is characterised by intermittent high spiking fever, skin rash, arthralgia, leukocytosis with neutrophilia, hyperferritinaemia, liver dysfunction, lymphadenopathy, and splenomegaly. 1,2 The diagnosis of AOSD is based on excluding other conditions, including autoimmune diseases, infectious diseases, and malignancies. The diagnosis is challenging and often delayed, as no specific markers are available in the clinic and laboratory.…”

Section: Introductionmentioning

confidence: 99%

“…The diagnosis is challenging and often delayed, as no specific markers are available in the clinic and laboratory. [1][2][3] Classic evanescent rash, which consists of evanescent, non-pruritic, salmon-pink macules and papules, is reported in up to 87% of patients with AOSD. 3 The rash disappears when the fever subsides.…”

Section: Introductionmentioning

confidence: 99%

“…4 Histologically, evanescent rash shows superficial perivascular inflammation with sparse neutrophil infiltration. 2,5 Evanescent rash has traditionally been a major and important diagnostic criterion for AOSD. [1][2][3] Another type of rash, persistent pruritic eruption (PPE), was first reported in patients with AOSD in the 1990s.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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

Qiao

Zhou

et al. 2019

Histopathology

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Autoinflammatory Diseases Presenting in the Skin

Lipsker,

Grattan,

Lovell

2024

Rook's Textbook of Dermatology

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Autoinflammatory diseases comprise a heterogeneous group of inherited monogenic and acquired polygenic multisystem disorders that may present in the skin and are therefore of importance to dermatologists. The inherited monogenic disorders typically present in childhood. Those that present with urticarial rashes are particularly important to differentiate from chronic urticaria since the pathogenesis, prognosis and treatment are completely different. Cryopyrin‐associated periodic syndrome should be suspected in children with a family history of chronic urticaria that does not respond to H 1 antihistamines and who have systemic symptoms of malaise and fever with persistently raised inflammatory indices. Autoinflammatory syndromes also may present with: unexplained fever, erysipelas‐like rash (familial Mediterranean fever), oedema (tumour necrosis factor receptor‐associated periodic syndrome), granulomatous dermatitis (Blau syndrome), an aseptic pustular dermatosis (deficiency of interleukin 1 receptor antagonist) or pyoderma gangrenosum (pyogenic sterile arthritis, acne and pyoderma gangrenosum), chilblain or acral erythematous atrophic or necrotic lesions in a toddler with neurological (Aicardi‐Goutières syndrome) or interstitial pneumonia (STING‐associated vasculopathy of infancy), lipoatrophy (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature), livedo, nodules (vasculitis) and stroke (deficiency of adenosine deaminase 2), aphtous stomatis with early‐onset bowel disease (haploinsufficiency of A20 or of RELA). Acquired autoinflammatory syndromes which present in adult life include Schnitzler syndrome and adult Still disease; the differential diagnosis includes urticarial vasculitis.

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Undifferentiated and Mixed Connective Tissue Disease and Dermatological Manifestations of Rheumatoid Disease

Laws

2024

Rook's Textbook of Dermatology

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Undifferentiated connective tissue disease (UCTD) and mixed connective tissue diseases (MCTD) include a spectrum of rheumatological signs and symptoms which overlap with traditional connective tissue diseases including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), systemic sclerosis (SSc) and dermatomyositis (DM) (the reader is directed to relevant chapters for discussion of SLE, DM and SSc). Describing the clinical features associated with UCTD and MCTD is inherently challenging due to the difficulties classifying overlap disorders precisely and the variable nomenclature in the literature. Despite this challenge the dermatologist has a key role in early recognition and diagnosis of affected patients. While the majority of rheumatological disease management focuses on controlling or reducing joint symptoms, several conditions have cutaneous features which may indicate active disease, impact on morbidity and negatively affect quality of life. In addition to UCTD and MCTD other rheumatic diseases which have significant cutaneous features include: rheumatoid arthritis, Sjogren syndrome, Still syndrome and rheumatic fever. This chapter provides an overview to these conditions with specific reference to cutaneous features. Appreciating the spectrum of dermatological manifestations associated with rheumatological disease and how these impact on the individual patient is important to promoting holistic, patient‐centred care. Close collaboration between dermatologists and rheumatologists is an essential prerequisite in supporting effective diagnosis and treatment of this complex cohort of patients.

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A comprehensive review on adult onset Still's disease

Cited by 311 publications

References 185 publications

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

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

Autoinflammatory Diseases Presenting in the Skin

Undifferentiated and Mixed Connective Tissue Disease and Dermatological Manifestations of Rheumatoid Disease

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