chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature

Also Known As:

POMP-related autoinflammation; POMP-related autoinflammation and immune dysregulation disease; PRAID

Networked: 15 relevant articles (2 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Immune System Diseases: 4321
- Immunologic Deficiency Syndromes: 91
  - chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature: 15

Related Diseases

1.	Bites and Stings (Sting)
2.	Chilblain lupus 1
3.	Pyoderma Gangrenosum
4.	Skin Diseases (Skin Disease)
5.	chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature

Experts

1.	Goldbach-Mansky, Raphaela: 3 articles (01/2022 - 03/2012)
2.	Lee-Kirsch, Min Ae: 3 articles (01/2022 - 10/2021)
3.	Poli, M Cecilia: 3 articles (01/2022 - 01/2018)
4.	Adang, Laura A: 2 articles (01/2022 - 01/2022)
5.	Aletaha, Daniel: 2 articles (01/2022 - 01/2022)
6.	Armangue, Thais: 2 articles (01/2022 - 01/2022)
7.	Barchus, Kathe: 2 articles (01/2022 - 01/2022)
8.	Brogan, Paul A: 2 articles (01/2022 - 01/2022)
9.	Cetin Gedik, Kader: 2 articles (01/2022 - 01/2022)
10.	Cordova, Devon R: 2 articles (01/2022 - 01/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature:

1.	baricitinibIBA 04/22/2023 - "This study evaluated the efficacy and safety of baricitinib (Janus kinase-1/2 inhibitor), in adult and pediatric Japanese patients with Nakajo-Nishimura syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (NNS/CANDLE), stimulator of interferon genes-associated vasculopathy with onset during infancy (SAVI), or Aicardi-Goutières syndrome (AGS). " 06/01/2021 - "Seventeen open-label studies assessing the efficacy of ruxolitinib, baricitinib, or tofacitinib reported variable responses in patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and related syndromes, stimulator of IFN genes [STING]-associated vasculopathy with onset in infancy (SAVI), familial chilblain lupus (FCh-L), gain-of-function mutations of STAT1 (GOF-STAT1), or Aicardi-Goutiéres syndrome. "
2.	tofacitinibIBA 03/01/2021 - "Successful treatment of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome with tofacitinib." 06/01/2021 - "Seventeen open-label studies assessing the efficacy of ruxolitinib, baricitinib, or tofacitinib reported variable responses in patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and related syndromes, stimulator of IFN genes [STING]-associated vasculopathy with onset in infancy (SAVI), familial chilblain lupus (FCh-L), gain-of-function mutations of STAT1 (GOF-STAT1), or Aicardi-Goutiéres syndrome. "
3.	ruxolitinibIBA 06/01/2021 - "Seventeen open-label studies assessing the efficacy of ruxolitinib, baricitinib, or tofacitinib reported variable responses in patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and related syndromes, stimulator of IFN genes [STING]-associated vasculopathy with onset in infancy (SAVI), familial chilblain lupus (FCh-L), gain-of-function mutations of STAT1 (GOF-STAT1), or Aicardi-Goutiéres syndrome. "
4.	Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link 05/01/2023 - "Janus kinase inhibitors are effective in CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) syndrome, anakinra in neutrophilic urticarial dermatosis, and intravenous immunoglobulin in resistant pyoderma gangrenosum. "
5.	Intravenous Immunoglobulins (IVIG)FDA Link 05/01/2023 - "Janus kinase inhibitors are effective in CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) syndrome, anakinra in neutrophilic urticarial dermatosis, and intravenous immunoglobulin in resistant pyoderma gangrenosum. "
6.	Janus Kinase InhibitorsIBA 05/01/2023 - "Janus kinase inhibitors are effective in CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) syndrome, anakinra in neutrophilic urticarial dermatosis, and intravenous immunoglobulin in resistant pyoderma gangrenosum. "
7.	InterferonsIBA 08/01/2015 - "Newer autoinflammatory diseases, such as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) are, however, driven by interferons. " 01/01/2022 - "Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. " 01/01/2022 - "Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. " 04/22/2023 - "This study evaluated the efficacy and safety of baricitinib (Janus kinase-1/2 inhibitor), in adult and pediatric Japanese patients with Nakajo-Nishimura syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (NNS/CANDLE), stimulator of interferon genes-associated vasculopathy with onset during infancy (SAVI), or Aicardi-Goutières syndrome (AGS). "
8.	Janus Kinase 1IBA 04/22/2023 - "This study evaluated the efficacy and safety of baricitinib (Janus kinase-1/2 inhibitor), in adult and pediatric Japanese patients with Nakajo-Nishimura syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (NNS/CANDLE), stimulator of interferon genes-associated vasculopathy with onset during infancy (SAVI), or Aicardi-Goutières syndrome (AGS). "
9.	Proteasome Endopeptidase Complex (Proteasome)IBA 01/01/2017 - "CANDLE syndrome (Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature) is a rare, genetic autoinflammatory disease due to abnormal functioning of the multicatalytic system proteasome-immunoproteasome. " 03/01/2012 - "Mutations in proteasome subunit β type 8 cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature with evidence of genetic and phenotypic heterogeneity." 10/26/2023 - "Panniculitis is a distinctive feature in proteasome-associated autoinflammatory syndromes (PRAAS), including chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome and Nakajo-Nishimura syndrome. " 01/01/2018 - "Biallelic or digenic loss-of-function variants in some of the proteasome subunits have been described as causing a primary immunodeficiency disease that manifests as a severe dysregulatory syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE). " 01/01/2022 - "Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. "
10.	InflammasomesIBA 12/02/2022 - "Combined with genetic testing results, 49 children with non-inflammasome related conditions were found, including 34 cases (69%) of Blau syndrome, 4 cases (8%) of tumour necrosis factor receptor-associated periodic syndrome, 4 cases (8%) of haploinsufficiency of A20, 2 cases (4%) of Majeed syndrome, 2 cases (4%) of pyogenic sterile arthritis, pyoderma gangrenosum, acne syndrome and 3 cases (6%) of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome. "

Therapies and Procedures

1.	Hematopoietic Stem Cell Transplantation 10/01/2021 - "Curative Treatment of POMP-Related Autoinflammation and Immune Dysregulation (PRAID) by Hematopoietic Stem Cell Transplantation."