Neutrophilic Dermatosis: A Case of Overlapping Syndrome with Monoclonal Antineutrophil Cytoplasmic Autoantibody Activity

Dermatology ◽  
1994 ◽  
Vol 189 (1) ◽  
pp. 69-71 ◽  
Author(s):  
P. Bayle ◽  
G. Laplanche ◽  
B. Gorguet ◽  
F. Oksman ◽  
S. Boulinguez ◽  
...  
Keyword(s):  
Neutrophilic Dermatosis ◽  
Antineutrophil Cytoplasmic Autoantibody

Sweet syndrome: Acute febrile neutrophilic dermatosis

1995 ◽  
Vol 14 (2) ◽  
pp. 173-178 ◽  
Author(s):  
W.P. Daniel Su ◽  
Debra L. Fett ◽  
Lawrence E. Gibson ◽  
Mark R. Pittelkow
Keyword(s):  
Neutrophilic Dermatosis ◽  
Sweet Syndrome ◽  
Acute Febrile Neutrophilic Dermatosis

Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with Waldenstroem's macroglobulinemia.

1990 ◽  
Vol 52 (5) ◽  
pp. 901-905
Author(s):  
Yoshihiro UMEBAYASHI ◽  
Hiromasa NAMEKI ◽  
Yoshio SAITO ◽  
Ryushi TAZAWA ◽  
Yuji OKA ◽  
...  
Keyword(s):  
Neutrophilic Dermatosis ◽  
Sweet’S Syndrome ◽  
Sweet's Syndrome ◽  
Acute Febrile Neutrophilic Dermatosis

scholarly journals OP0286 PROTEOMICS AND RNA SEQUENCING APPROACHES HIGHLIGHT THE ROLE OF ENDOTHELIAL CELL DYSREGULATION IN IL-1 AND IFN MEDIATED AUTOINFLAMMATORY DISEASES, NOMID AND CANDLE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 178-179
Author(s):  
S. Alehashemi ◽  
M. Garg ◽  
B. Sellers ◽  
A. De Jesus ◽  
A. Biancotto ◽  
...  
Keyword(s):  
Endothelial Cell ◽  
Whole Blood ◽  
Cell Activation ◽  
Immune Cell ◽  
Differentially Expressed ◽  
Neutrophilic Dermatosis ◽  
Healthy Controls ◽  
Differentially Expressed Proteins ◽  
Rna Seq ◽  
Before And After

Background:Systemic Autoinflammatory diseases present with sterile inflammation. NOMID (Neonatal-Onset Multisystem Inflammatory Disease) is caused by gain-of-function mutations inNLRP3and excess IL-1 production, presents with fever, neutrophilic dermatosis, aseptic meningitis, hearing loss and eye inflammation; CANDLE (Chronic Atypical Neutrophilic Dermatosis, Lipodystrophy and Elevated Temperature) is caused by loss-of-function mutations in proteasome genes that lead to type-1 interferon signaling, characterized by fever, panniculitis, lipodystrophy, cytopenia, systemic and pulmonary hypertension and basal ganglia calcification. IL-1 blockers are approved for NOMID and JAK-inhibitors show efficacy in CANDLE treatment.Objectives:We used proteomic analysis to compare differentially expressed proteins in active NOMID and CANDLE compared to healthy controls before and after treatment, and whole blood bulk RNA seq to identify the immune cell signatures.Methods:Serum samples from active NOMID (n=12) and CANDLE (n=7) before and after treatment (table 1) and age matched healthy controls (HC) (n=7) were profiled using the SomaLogic platform (n=1125 proteins). Differentially expressed proteins in NOMID and CANDLE were ranked after non-parametric tests for unpaired (NOMIDp<0.05, CANDLE,p<0.1) and paired (p<0.05) analysis and assessed by enriched Gene Ontology pathways and network visualization. Whole blood RNA seq was performed (NOMID=7, CANDLE=7, Controls =5) and RPKM values were used to assess immune cells signatures.Table 1.Patient’s characteristicsNOMIDN=12, Male =6CANDLEN=7, Male =6AgeMedian (range)12 (2, 28)16 (3, 20)Ethnicity%White (Hispanic)80 (20)100 (30)GeneticsNLRP3mutation(2 Somatic, 10 Germline)mutations in proteasome component genes(1 digenic, 6 Homozygous/compound Heterozygous)Before treatmentAfter treatmentBefore treatmentAfter treatmentCRPMedian (range) mg/L52 (16-110)5 (0-23)5 (0-101)1 (0-4)IFN scoremedian (range)0NA328 (211-1135)3 (0-548)Results:Compared to control, 205 proteins (127 upregulated, 78 downregulated) were significantly different at baseline in NOMID, compared to 163 proteins (101 upregulated, and 62 downregulated) in CANDLE. 134 dysregulated proteins (85 upregulated, 49 downregulated) overlapped in NOMID and CANDLE (Figure 1). Pathway analysis identified neutrophil and monocyte chemotaxis signature in both NOMID and CANDLE. NOMID patients had neutrophilia and active neutrophils. CANDLE patients exhibited active neutrophils in whole blood RNA. Endothelial cell activation was the most prominent non-hematopoietic signature and suggest distinct endothelial cell dysregulation in NOMID and CANDLE. In NOMID, the signature included neutrophil transmigration (SELE) endothelial cell motility in response to angiogenesis (HGF, VEGF), while in CANDLE the endothelial signatures included extracellular matrix protein deposition (COL8A) suggesting increased vascular stiffness. CANDLE patients had higher expression of Renin, 4 out of 7 had hypertension, NOMID patients did not have hypertension. Treatment with anakinra and baricitinib normalized 143 and 142 of dysregulated proteins in NOMID and CANDLE respectively.Conclusion:Differentially expressed proteins in NOMID and CANDLE are consistent with innate immune cell activation. Distinct endothelial cell signatures in NOMID and CANDLE may provide mechanistic insight into differences in vascular phenotypes. Treatment with anakinra and Baricitinib in NOMID and CANDLE leaves 30% and 13% of the dysregulated proteins unchanged.Acknowledgments:This work was supported by Intramural Research atNational Institute of Allergy Immunology and Infectious Diseases of National Institutes of Health, Bethesda, Maryland, the Center of Human Immunology and was approved by the IRB.Disclosure of Interests:None declared

Neutrophilic Dermatosis of Hands as Oncological Finding: Importance of Follow-Up

ORL ◽  
2021 ◽  
pp. 1-3
Author(s):  
Jérôme R. Lechien ◽  
Daphné Delplace ◽  
Mohamad Khalife ◽  
Sven Saussez
Keyword(s):  
Head And Neck Cancer ◽  
Head And Neck ◽  
Neck Cancer ◽  
Paraneoplastic Syndrome ◽  
Tobacco Consumption ◽  
Neutrophilic Dermatosis ◽  
Pharyngeal Cancer ◽  
Head And Neck Malignancy ◽  
Neck Malignancy

Neutrophilic febrile dermatosis (NFD) is a rare paraneoplastic syndrome that may be found in patients with head and neck cancer. NFD may appear before the neoplasia and may only concern the dorsal faces of the hands. This article reports the NFD findings of a patient with pharyngeal cancer, which was developed 2 years after the occurrence of NFD. The development of NFD in patient with alcohol and tobacco consumption should lead otolaryngologists and dermatologists to suspect head and neck malignancy. In cases of normal otolaryngological examination, patients have to be followed.

scholarly journals Sweet Syndrome and Secondary Syphilis in a Person with Acute Necrotizing Tonsillitis

2021 ◽  
Vol 13 (1) ◽  
pp. 216-221
Author(s):  
Joseph Mishal ◽  
Igor Viner ◽  
Alexandro Livoff ◽  
Shlomo Maayan ◽  
Eli Magen
Keyword(s):  
Unusual Case ◽  
Treponema Pallidum ◽  
Cervical Lymphadenopathy ◽  
Secondary Syphilis ◽  
Neutrophilic Dermatosis ◽  
Sweet Syndrome ◽  
Acute Febrile Neutrophilic Dermatosis ◽  
Acute Necrotizing ◽  
The Right ◽  
Syphilis Serology

Syphilis has received its classical designation as one of “the great imitators,” reflecting a wide variety of symptoms and presentations, which can cause difficulties in diagnosis. Here we report an unusual case of secondary syphilis in a person with acute necrotizing tonsillitis and Sweet syndrome. A 33-year-old female presented with fever, bilateral cervical lymphadenopathy, tonsillar enlargements with ulcerated pus-filled lesions on the right tonsil, and multiple pseudovesicular, mammillated, edematous plaques on her neck, face, and extremities. Syphilis serology was positive and a skin biopsy demonstrated a neutrophil-rich dermatitis characteristic of Sweet syndrome. The association of <i>Treponema pallidum</i> infection with Sweet syndrome may be a coincidence; nevertheless, our case serves as a reminder that secondary syphilis should remain in the differential diagnosis of the acute febrile neutrophilic dermatosis.

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