Table 3.
Literature review of the management of MGUS-related skin disorders.
| Author/Year | Patients number | MGUS type | Type of skin involvement | Type of therapy | Outcome | MGUS response |
|---|---|---|---|---|---|---|
| de Chambrun 2017 (63) | 57 | IgG-κ, IgG-λ | Clarkson disease | -IVIG (48) -Terbutaline (22) Median follow up of 5.1 years |
-Complete/partial response -No response |
-Lower IgG levels -No response -MM (5) |
| Kapoor 2010 (64) | 19 | IgG | Clarkson disease | -Methylxanthines (23), terbutaline (21), leukotriene inhibitor (10) -Zafirlukast + lisinopril (2) Median follow up of 4.9 years |
-No response -Partial response |
N/A |
| Wood 2009 (65) | 17 | κ, γ light chains | Necrobiotic xanthogranuloma | -Chemotherapy (2) -Chlorambucil + prednisolone (4) -Melphalan + prednisolone (1) -Dexamethasone (1) -Thalidomide + prednisolone (1) -Rituximab (2) -Intralesional corticosteroids + topical immunomodulators (2) |
-Partial response (2) -Complete response (2) -Partial response (1) -Partial response -2 years of remission -Complete response (1) -No response |
N/A |
| Higgins 2016 (66) | 28 | IgG-κ, IgG-λ, IgM-κ |
Necrobiotic xanthogranuloma | -AHCT (3) -Chlorambucil ± corticosteroids (5) -FCR (1) -Melphalan + corticosteroids (3), VDD (2), antibiotics (3), cyclophosphamide + corticosteroids (4) -Corticosteroids (11) -Rituximab (6) -IVIG (4) -Thalidomide ± corticosteroids (11) -Lenalidomide ± corticosteroids (11) -Bortezomib ± corticosteroids (4) -BLD (1) |
-Complete response (2) -Complete response (2) -Complete response (1) -No response -Complete response (4) -Complete response (1) -Complete response (2) -Complete response (4) -Complete response (7) -Complete response (1) -Complete response (1) |
N/A |
| Szalat 2011 (67) | 4 | IgG-κ, IgG-λ, IgM-κ |
Necrobiotic xanthogranuloma | -Thalidomide + bortezomib -Corticosteroids + chlorambucil -Chlorambucil + rituximab + fludarabine + cyclophosphamide + thalidomide + dexamethasone |
-Partial response (1) -Partial response (2) -Complete response (1) |
N/A |
| Donato 2006 (68) | 7 | IgG-κ, IgG-λ | Scleromyxedema | AHCT | Complete (5), partial response (2) | N/A |
| Kreuter 2005 (69) | 4 | IgG-κ, IgG-λ | Scleromyxedema | -IVIG (4), cyclophosphamide (1) -Dexamethasone (3) -Bortezomib (1) -Phototherapy, acitretin, methotrexate, thalidomide |
-Partial response, recurred -Complete response, recurred -Complete response (1) -No response |
N/A |
| Mahevas 2020 (70) | 31 | IgG-κ, IgG-λ | Scleromyxedema | -IVIG (21) -IVIG + corticosteroids (10) -IVIG + lenalidomide (1) -IVIG + thalidomide (1) -Lenalidomide (3) -Thalidomide (3) -Acitretin (2) -Corticosteroids (3) -Melphalan + dexamethasone (2) -Methotrexate (1) |
-Complete or partial response -Complete or partial response -Complete response -Partial response -Weak response -Weak response -No response -Complete or no response -Weak response -No response |
-Complete response with IVIG |
| Rongioletti 2013 (71) | 25 | IgG-κ, IgG-λ | Scleromyxedema | -IVIG (11) -IVIG + lenalidomide (1) -IVIG + corticosteroids (1) -Thalidomide (1) -Acitretin (1) -Mycophenolate (1) -Prednisolone + hydroxychloroquine (1) -Prednisolone + thalidomide (1) -Corticosteroids (2) -Mycophenolate (1) -Other therapies |
-Complete/partial response (3/6) -Complete response (1) -Complete response (1) -Partial response (1) -Partial response (1) -Partial response (1) -Partial response (1) -Partial response (1) -No response (2) -No response (1) -No response |
N/A |
| Terpos 2012 (72) | 13 | IgM-κ, IgM-λ, IgG-κ, IgG-λ | Schnitzler syndrome | -Perfloxacin (8) -Anakinra (7) |
-Complete response (5) -Complete response (7) |
N/A |
| Sokumbi 2012 (73) | 20 | IgM-κ, IgM-λ, IgG-κ, IgG-λ | Schnitzler syndrome | -Corticosteroids (13) -Rituximab (3), -Cyclophosphamide (1) -Anakinra (2) |
-Partial response (11) -Partial response (2) -Partial response (1) -Partial response (1) |
Malignant transformation (9) |
| Gusdorf 2017 (74) | 25 | IgM, IgG | Schnitzler syndrome | -Anakinra (29) | -Complete response (23) | N/A |
| Neel 2014 (75) | 42 | IgM-κ | Schnitzler syndrome | -Anakinra or canakinumab | -Complete response (29) at 36 months of follow up | N/A |
AHCT, autologous hematopoietic cell transplantation; BLD, bortezomib + lenalidomide + dexamethasone; FCR, fludarabine + cyclophosphamide + rituximab; FLD, fludarabine; MM, multiple myeloma; N/A, not available; R-CHOP, cyclophosphamide, hydroxydaunorubicin, vincristine, prednisolone; VDD, vincristine + doxorubicin + dexamethasone.