Characteristics of 33 scleromyxedema patients
| . | Results . |
|---|---|
| Patients | |
| Sex ratio (M/F) | 1.06 (17/16) |
| Age, mean ± SD, y | 55.4 ± 13.6 |
| Follow-up, mean ± SD, y | 4.3 ± 2.39 |
| Death | 1 (3) |
| Cutaneous manifestations | 33 (100) |
| Papular eruption/pruritus | 33 (100)/18 (54) |
| Sclerodermoid eruption/leonine facies | 30 (90)/13 (39) |
| Depilation/alopecia | 10/22 (45)/4 (12) |
| Purpura/livedo racemosa/cutis laxa–like syndrome/skin necrosis | 1 (3)/3 (9)/3 (9)/1 (3) |
| Extracutaneous manifestations | 22 (67) |
| Hematological involvement | 33 (100) |
| MG/MGUS/hematologic malignancy | 33 (100)/30 (91)/4 (12) |
| Neurological involvement | 18 (54) |
| Central nervous system involvement/DNS | 9 (27)/6 (18) |
| Carpal tunnel syndrome | 12 (36) |
| Sensitive polyneuropathy | 1 (3) |
| Cardiac and vascular involvement | 5 (15) |
| Ischemic/mucinous cardiopathy | 3 (9)/2 (6) |
| Arterial hypertension | 11 (33) |
| Pulmonary involvement | 3 (9) |
| Obstructive syndrome | 3 (9) |
| Interstitial pneumopathy | 1 (3) |
| Musculoskeletal involvement | 9 (27) |
| Myositis/myalgia | 2 (6)/2 (6) |
| Arthralgia/arthritis | 9 (27)/1 (3) |
| Gastrointestinal involvement (dysphagia) | 2 (6) |
| Head and neck involvement (dysphonia) | 1 (3) |
| Ophthalmic (retrobulbar optic neuritis) | 1 (3) |
| Laboratory findings | |
| MG/biclonal gammopathy | 33 (100)/1 (3) |
| Serum IgG level, g/L | 4.5 ± 3 |
| κ or λ light chain in monoclonal Ig | 10/33 (29) or 24/33 (73) |
| Skin histological findings | 33 (100) |
| Dermal mucinosis/fibroblast proliferation/fibrosis | 33 (100) |
| Granuloma annulare form: histiocytic infiltrate (CD68+ and CD163+) | 10 (27) |
| . | Results . |
|---|---|
| Patients | |
| Sex ratio (M/F) | 1.06 (17/16) |
| Age, mean ± SD, y | 55.4 ± 13.6 |
| Follow-up, mean ± SD, y | 4.3 ± 2.39 |
| Death | 1 (3) |
| Cutaneous manifestations | 33 (100) |
| Papular eruption/pruritus | 33 (100)/18 (54) |
| Sclerodermoid eruption/leonine facies | 30 (90)/13 (39) |
| Depilation/alopecia | 10/22 (45)/4 (12) |
| Purpura/livedo racemosa/cutis laxa–like syndrome/skin necrosis | 1 (3)/3 (9)/3 (9)/1 (3) |
| Extracutaneous manifestations | 22 (67) |
| Hematological involvement | 33 (100) |
| MG/MGUS/hematologic malignancy | 33 (100)/30 (91)/4 (12) |
| Neurological involvement | 18 (54) |
| Central nervous system involvement/DNS | 9 (27)/6 (18) |
| Carpal tunnel syndrome | 12 (36) |
| Sensitive polyneuropathy | 1 (3) |
| Cardiac and vascular involvement | 5 (15) |
| Ischemic/mucinous cardiopathy | 3 (9)/2 (6) |
| Arterial hypertension | 11 (33) |
| Pulmonary involvement | 3 (9) |
| Obstructive syndrome | 3 (9) |
| Interstitial pneumopathy | 1 (3) |
| Musculoskeletal involvement | 9 (27) |
| Myositis/myalgia | 2 (6)/2 (6) |
| Arthralgia/arthritis | 9 (27)/1 (3) |
| Gastrointestinal involvement (dysphagia) | 2 (6) |
| Head and neck involvement (dysphonia) | 1 (3) |
| Ophthalmic (retrobulbar optic neuritis) | 1 (3) |
| Laboratory findings | |
| MG/biclonal gammopathy | 33 (100)/1 (3) |
| Serum IgG level, g/L | 4.5 ± 3 |
| κ or λ light chain in monoclonal Ig | 10/33 (29) or 24/33 (73) |
| Skin histological findings | 33 (100) |
| Dermal mucinosis/fibroblast proliferation/fibrosis | 33 (100) |
| Granuloma annulare form: histiocytic infiltrate (CD68+ and CD163+) | 10 (27) |
Unless otherwise noted, data are n (%).