Proteasome-associated Autoinflammatory Syndrome (PRAAS): aka Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature Syndrome (CANDLE), Nakajo-Nishimura Syndrome (NNS), or Japanese Autoinflammmatory Syndrome (JMP)
PRAAS/CANDLE/JMP, also subclasses: PSMB8-PRAAS, PSMB4/PSMB9-PRAAS, PSMA3/PSMB8-PRAAS
Proteasome-Associated Autoinflammatory Syndrome (PRAAS)
PSMB8; also PSMB4, PSMB9, PSMA3, POMP 
Unknown, but rare.
Annular cutaneous plaques with residual purpura. Lipodsystrophy first noted on the face and around joints, and the limbs. Lips swell with flares. Persistently swollen, violaceous (purple-red) eyelids.  
Some have frequent otitis and/or recurrent sinusitis. 
Diarrhea or loose bowels with flares. Hepatomegaly and splenomegaly (hepatosplenomegaly) with elevated liver enzymes. Delayed or slow growth. At risk for being underweight, or failure to thrive. At risk for multi-organ failure and death. ,  Large abdomen from an enlarged liver and spleen is possible.
Joints bones muscles cartilage:
Joint contractures, muscle atrophy, panniculitis induced lipodystrophy, myositis, fatigue and malaise. Inflamed nose and ear cartilage (chondritis). Short stature. Delayed growth in height and weight is common.  
Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature Syndrome,CANDLE, Proteasome associated autoinflammatory syndromes, PRAAS, Nakajo-Nishimura Syndrome, NNS, or Japanese autoinflammmatory syndrome, JMP, periodic fever, recurrent fever, chronic inflammation, cutaneous plaques, annular cutaneous plaques, residual purpura, lipodsystrophy, violaceous eyelids, purple eyelids,violet eyelids, violaceous, aseptic meningitis, growth delay, developmental delay, mental delay, swollen lips, nodular episcleritis, episcleritis, keratitis, periorbital edema, eyelid swelling, lip swelling, clubbing, cardiac arrythmia, cardiomyopathy, Hepatomegaly, splenomegaly, hepatosplenomegaly, failure to thrive, liver enzymes, multi-organ failure, loss of body fat, large abdomen, swollen abdomen, abdominal swelling, lymphadenopathy, joint contractures, muscle atrophy, panniculitis induced lipodystrophy, panniculitis, myositis, fatigue, inflamed nasal cartilage, ear cartilage, cartilage, chondritis, short stature, cartilage, nose, red nasal bridge, anemia, triglycerides, abdominal pain, pain, muscle pain, joint pain, lymph, lymph node
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