Table 1. List of Clinical Features and Initial Therapies of 21 Japanese Patients.
| Patient No./Sex/Decade of Age at Onset | Cutaneous Manifestation | Localization | Neuropathy | Arthropathy | Initial Therapy | Follow-up, mo | Current Status/Therapy | ||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Lower Leg | Thigh | Trunk | Arm | Head/Neck | |||||||
| 1/M/50s | Indurated erythema; livedo; cutaneous ulcer; purpura | + | − | − | − | − | + | − | Prednisone, 30 mg/d | 374 | Death due to brain hemorrhage |
| 2/M/30s | Indurated erythema; livedo; cutaneous ulcer; purpura | + | + | + | + | + | − | +a | Ticlopidine hydrochloride, cilostazol, and aspirin | 335 | Prednisone, 5 mg/d, and warfarin |
| 3/F/20s | Indurated erythema; livedo | + | − | − | + | − | − | + | Prednisone, 45 mg/d | 296 | PSL4 |
| 4/F/10s | Indurated erythema | + | − | − | + | + | − | + | Intravenous pulse methylprednisolone and prednisone, 45 mg/d | 243 | Complete response |
| 5/F/60s | Indurated erythema; livedo; purpura | + | + | + | − | − | + | − | Prednisone, 50 mg/d | 49 | Death due to infection |
| 6/F/30s | Indurated erythema | − | − | + | + | − | − | + | NSAIDs | 31 | Complete response |
| 7/F/50s | Indurated erythema; livedo; purpura | + | + | − | + | − | − | + | Prednisone, 20 mg/d | 74 | Complete response |
| 8/M/40s | Indurated erythema; livedo; cutaneous ulcer | + | − | − | − | − | + | − | Prednisone, 30 mg/d, aspirin, sarpogrelate hydrochloride and colchicine, and azathioprine sodium | 103 | Prednisone, 8 mg/d, endoxan, and warfarin |
| 9/F/10s | Indurated erythema; livedo | + | − | − | − | − | − | + | NSAIDs | 28 | Complete response |
| 10/F/50s | Indurated erythema; livedo | + | + | + | + | − | + | + | Prednisone, 40 mg/d, endoxan pulse, and diaminodiphenyl sulfone benzoate, 50 mg/d | 95 | Betamethasone, 1.8 mg/d, aspirin, and tocopherol acetate |
| 11/F/10s | Indurated erythema; livedo | + | − | − | − | − | − | − | Aspirin | 78 | Complete response |
| 12/F/40s | Indurated erythema; livedo | + | − | − | − | − | + | − | Prednisone, 20 mg/d, and aspirin | 64 | Aspirin |
| 13/M/60s | Indurated erythema; livedo | + | − | − | − | − | − | + | Prednisone, 15 mg/d, and aspirin | 27 | Prednisone, 7 mg/d, and aspirin |
| 14/M/60s | Indurated erythema; livedo | + | + | − | + | − | + | − | Prednisone, 30 mg/d, aspirin, and clopidogrel bisulfate | 42 | Prednisone, 5 mg/d, aspirin, and clopidogrel bisulfate |
| 15/F/60s | Indurated erythema; livedo; cutaneous ulcer | + | + | + | − | − | + | + | Prednisone, 10 mg/d | 25 | Death due to cancer |
| 16/F/20s | Livedo | + | + | − | + | − | + | + | Prednisone, 20 mg/d, aspirin, and clopidogrel bisulfate | 28 | Complete response |
| 17/F/20s | Indurated erythema; livedo | + | − | − | - | − | − | − | Prednisone, 20 mg/d, aspirin, and clopidogrel bisulfate | 19 | Aspirin and clopidogrel bisulfate |
| 18/F/40s | Indurated erythema; livedo | + | + | − | + | − | + | − | Aspirin and clopidogrel bisulfate | 19 | Aspirin and clopidogrel bisulfate |
| 19/F/60s | Indurated erythema | − | − | − | + | − | + | − | Prednisone, 10 mg/d, aspirin, and clopidogrel bisulfate | 11 | Prednisone, 7.5 mg/d, aspirin, and clopidogrel bisulfate |
| 20/F/70s | Indurated erythema; livedo | + | − | − | − | − | − | − | Prednisone, 20 mg/d | 10 | Prednisone, 3 mg/d, and asprin |
| 21/F/50s | Livedo | + | − | − | + | − | + | − | Prednisone, 30 mg/d, endoxan, aspirin, and clopidogrel bisulfate | 8 | Aspirin and clopidogrel bisulfate |
Abbreviations: NSAIDs, nonsteroidal anti-inflammatory drugs; minus sign, absent; plus sign, present.
a
Indicates marked hydrarthrosis of bilateral knees and ankles joints.