|
Author | Age | Sex | Location | Previous symptoms | Autoimmune complications | Time to diagnosis | Treatment | Survival | Cause of death |
|
Park et al. [10] | 40 | W | Skin | High fever, proximal muscle weakness, multiple skin plaques with bullae and serous discharge | Dermatomyositis and hemophagocytic syndrome | 24 months | CHOP → steroid, antibiotics, gamma globulin, oral cyclosporin | 7 days | Hepatic failure, renal failure, pancytopenia, massive pleural effusion |
Kim et al. [2] | 64 | M | Skin | Painful swelling and redness of the left upper arm | Cellulitis or fasciitis | 5 months | Antibiotics → L-asparagine chemotherapy | n.r. | n.r. |
Chan et al. [11] | 68 | W | Muscle | Forearm swelling and bilateral facial swelling | Polymyositis | 4-5 weeks | Prednisolone | Few days | Fulminant hemophagocytic syndrome |
Spadigam et al. [12] | 49 | M | Skin | Intermittent fever, nasal stuffiness, epistaxis and hemifacial pain, nasolabial lesion | Inflammatory myofibroblast | 3 months | Surgery | 2 weeks | Postoperative complications |
Fei et al. [13] | 83 | W | Lung | Skin ulceration and intermittent fever | Panniculitis | 12 months | Antibiotics → patient refused treatment | 35 days | Multiorgan failure |
Chow et al. [9] | 27 | W | Skin | Intermittent fever, occasional night sweats, nasal congestion and hoarseness, skin nodules | Panniculitis or sarcoidosis | 3 months | Antiviral/antibiotic → CHOP → ICE | 7 months | Secondary hemophagocytic syndrome |
Current | 56 | M | Skin | Bilateral nasal respiratory insufficiency, anterior septal perforation, skin lesions | Myositis and uveitis | 13 months | CHOP → SMILE → BMT | 30 months | Alive |
|