PedAM
Pediatric Disease Annotations & Medicines
| Disease | lipodystrophy |
| Phenotype | C0011603|dermatitis |
| Sentences | 2 |
| PubMedID- 23971037 | Despite demonstration of the great efficacy of il-1 blocking therapy in many aids, revealing innate immune system workings and the key role of il-1 in autoinflammatory attacks, new aids have recently been recognized as unresponsive to il-1 antagonists, including the proteasome-related “chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperature” syndrome, or candle syndrome, which was initially described in different areas of japan and named nakajo-nishimura syndrome [47]. |
| PubMedID- 22288582 | Despite the tremendous success of il-1 blocking therapy, the use of these agents in a wider spectrum of autoinflammatory conditions has uncovered disease subsets that are not responsive to il-1 blockade, including the recently discovered proteasome-associated autoinflammatory syndromes such as chronic atypical neutrophilic dermatitis with lipodystrophy and elevated temperatures (candle), japanese autoinflammatory syndrome with lipodystrophy (jasl), nakajo-nishimura syndrome (nns) and joint contractures, muscle atrophy, panniculitis induced lipodystrophy (jmp), and urge the continued quest to characterize additional dysregulated innate immune pathways that cause autoinflammatory conditions. |
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