An atypical persistent eruption of adult‐onset Still's disease with neutrophilic urticarial dermatosis‐like dermal features: A case report and review of the literature

Nassereddine, Hussein; Fite, C.; Kottler, Diane; Descamps, V.; Couvelard, Anne; Deschamps, Lydia

doi:10.1111/cup.13320

Cited by 8 publications

(4 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It has a distinct reaction pattern associated with systemic diseases such as adult-onset Still disease, systemic lupus erythematosus, Schnitzler syndrome, and autoinflammatory diseases (ie, cryopyrin-associated periodic syndromes [CAPS]). 1,2,4,5 Unlike other neutrophilic dermatoses, such as Sweet syndrome, NUD histologically lacks dermal edema. 3 Although leukocytoclasia may be present, vessel wall alteration is not seen.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Urticaria and a rare mutation: An unusual case of neutrophilic urticarial dermatosis

et al. 2020

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

“…Notably, it lacks leukocytoclastic vasculitis. [1][2][3][4] In this case, we discuss a 59-year-old white woman with NUD who harbored a novel heterozygous mutation for NLR Family Pyrin Domain Containing 3 (NLRP3) gene. This gene provides instructions for the protein synthesis of cryopyrin.…”

Section: Introductionmentioning

confidence: 99%

Urticaria and a rare mutation: An unusual case of neutrophilic urticarial dermatosis

et al. 2020

View full text Add to dashboard Cite

“…128 AOSD patients had a significantly higher serum ferritin concentration than sJIA patients during the active disease phase (p < 0.01). 127,128 To identify AOSD patients, the Yamaguchi's criteria are widely used being a sensitive tool (sensitivity 96.2% and specificity 92.1%) for diagnosis and classification of AOSD 129 (Table 2), followed by Cush's criteria (sensitivity 80.6%) [130][131][132] and Fautrel's criteria (sensitivity 80.6% and specificity 98.5%) (Table 2). [133][134][135] Recently, Crispín's clinical scale (sensitivity 76.9% and specificity 98%) (Table 2) has also been adopted.…”

Section: Clinical Manifestations and Diagnosismentioning

confidence: 99%

New insights on multigenic autoinflammatory diseases

Efthimiou¹,

Petryna

Nakasato

et al. 2022

Therapeutic Advances in Musculoskeletal

View full text Add to dashboard Cite

Autoinflammatory diseases are disorders of the innate immune system, which can be either monogenic due to a specific genetic mutation or complex multigenic due to the involvement of multiple genes. The aim of this review is to explore and summarize the recent advances in pathogenesis, diagnosis, and management of genetically complex autoinflammatory diseases, such as Schnitzler’s syndrome; adult-onset Still’s disease; synovitis, acne, pustulosis, hyperostosis, osteitis syndrome/chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis; Adamantiades-Behçet’s disease; Yao syndrome; and periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. The PubMed database was screened for relevant articles using free text words and specific search strings. The search was limited to English-language articles, reporting the results of studies in humans, published through March 2021. Evidence from literature suggest that these rare multigenic autoinflammatory diseases can present with different clinical features and the diagnosis of these diseases can be challenging due to a combination of nonspecific manifestations that can be seen in a variety of other conditions. Diagnostic delays and disease complications may occur due to low disease awareness and the lack of pathognomonic markers. The pathogeneses of these diseases are complex and in some cases precise pathogenesis is not clearly understood. Conventional treatments are commonly used for the management of these conditions, but biologics have shown promising results. Biologics targeting proinflammatory cytokines including IL-1, IL-6, TNF-α, IL-17A and IL-18 have been shown to ameliorate signs and symptoms of different multigenic autoinflammatory diseases.

show abstract

“…She met all four major criteria and three of four minor criteria for the diagnosis of AOSD (12), while active infections and malignancy were excluded (Table 1). Punch biopsy of the skin rash was consistent with neutrophilic urticarial dermatosis (Figure 2), which is commonly seen in AOSD (13)(14)(15). Three days after admission the decision was made to start prednisone 60 mg/day for AOSD.…”

Section: Case Presentationmentioning

confidence: 99%

Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still’s disease: case report and review of the literature

Gabr¹,

Liu²,

Mian³

et al. 2020

Ann Transl Med

View full text Add to dashboard Cite

Here, we present a 22-year-old female patient with adult-onset Still's disease (AOSD) who was newly diagnosed in the setting of secondary macrophage activation syndrome (MAS), a rare, life-threatening inflammatory disease with 50% mortality due to multi-organ failure. She met the diagnostic criteria of AOSD and MAS, while genetic testing excluded primary causes of MAS. She had high fevers, anemia, thrombocytopenia, splenomegaly, hematophagocytosis, and elevated serum ferritin (37,950 ng/mL) and CD25 levels (11,870 pg/mL), which remained unresponsive to corticosteroids and anakinra. Her serum interferon gamma (IFN-γ) levels were elevated (7 pg/mL). She was markedly responsive to IFN-γ blockade with emapalumab that eliminated her fevers and all MAS-associated laboratory abnormalities. This report provides initial evidence for therapeutic efficacy for IFN-γ blockade in AOSD and secondary MAS.

show abstract

An atypical persistent eruption of adult‐onset Still's disease with neutrophilic urticarial dermatosis‐like dermal features: A case report and review of the literature

Cited by 8 publications

References 25 publications

Urticaria and a rare mutation: An unusual case of neutrophilic urticarial dermatosis

Urticaria and a rare mutation: An unusual case of neutrophilic urticarial dermatosis

New insights on multigenic autoinflammatory diseases

Successful treatment of secondary macrophage activation syndrome with emapalumab in a patient with newly diagnosed adult-onset Still’s disease: case report and review of the literature

Contact Info

Product

Resources

About