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Table 2 Literature review of patients with myeloma and scleredema

From: Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature

Year Age (years) Sex Clinical features Myeloma Bone marrow plasma cells Myeloma investigations Treatment Prognosis Reference
1974 37 M 2 years of disease MM (IgG-κ)   Urine Bence-Jones-positive Chemotherapy Good response [3]
1984 69 M   Smoldering myeloma (IgG-κ)      [2]
1987 64 M Rapid onset of skin involvement of face and chest; 7 years of little change; rapid progression to involvement of the shoulders, arms, back, abdomen, and thighs over 2 years MM (IgG-κ); IgG concentration of 4500 mg/dl   Urine immunoelectrophoresis IgG-κ monoclonal proteinuria, anemia; skeletal survey normal Prednisolone 80 mg/day and melphalan 14 mg/day, given for 4 days and repeated monthly From the sixth cycle, clear clinical evidence of softening of the skin and improved joint mobility [4]
1988 72 F Symmetrical woody induration of face, neck, chest, shoulders, upper back, chest, abdomen, thigh over 2 years MM (IgG-κ); IgG concentration of 2900 mg/dl   Osteolytic lesions, Bence-Jones-negative Melphalan Recovery in 1.5 years [14]
1988 76 F Stiffening of the skin of upper trunk, arms, neck, face over 24 h with woody induration MM (IgA-κ); IgA 814 mg/dl 20% Urine κ-light chains, multiple lytic lesions on skull x-ray IV cyclophosphamide 750 mg over 3 days, prednisolone 30 mg daily for 3 days; six pulses; each pulse over interval of 3 weeks Complete recovery of scleredema and remission of MM [15]
1988 62 F Thickening of skin over 23 years with induration over face, neck, thorax, arms with loss of movements of the underlying joints MM (IgG-λ); IgG 2500 mg/dl 40% anemia, urine Bence-Jones λ-light chains, cardiomyopathy Pulse chemotherapy Death with sepsis [9]
1992 46 M Stiffening of face, neck, back, shoulders, chest, arms, hands, fingers with woody induration Smoldering myeloma (IgG-λ) 28%     [6]
1995 74 M Marked induration of the skin of neck, shoulders, upper chest, back, and upper arms over 15 years MM; IgA-κ; IgA, 2530 mg/dl 67% Anemia, Bence-Jones protein (κ-light chain), osteolytic lesions Chemotherapy with intravenous vincristine, cyclophosphamide, oral melphalan, prednisone 90 mg; six cycles at 3-weekly intervals After the sixth chemotherapy cycle, myeloma was in remission; marked improvement of the skin [7]
1997 56 F Scleredema over the last 6 years with acanthosis nigricans MM; IgA-κ    Melphalan and prednisolone Recovery [10]
2000 63 F thickening of the skin on the face, neck, shoulders, arms, and upper torso while receiving treatment for MM MM; IgG-κ; IgG 9600 mg/dl 80% Urine IgG-κ light chain protein, anemia, lytic lesions in the skull Melphalan 10 mg/day, cyclophosphamide 200 mg/day, prednisolone 60 mg/day given for 5 days, vincristine 2 mg given on the first day; six courses with a 3-week gap Softening of the skin observed from sixth treatment cycle [8]
2001 70 F over 12 months of period, she had developed a progressive induration and stiffness of the skin of her face, neck, shoulders, and upper aspect of her arms MM; IgA-λ; IgA, 1830 mg/dl 38% Urine Bence-Jones protein Oral melphalan, oral prednisone; six pulses, each pulse at an interval of 1 month Clinical evidence of softening of the involved skin observed [16]
2008 28 M Eight years of progressive diffuse cutaneous thickening of face, trunk, arms, and thighs MM; IgA-κ 55% Anemia, myelofibrosis Thalidomide, dexamethasone Improvement in the texture of the skin [5]
2013 60 M Symmetric, nonpitting swelling of the face, neck, trunk, and upper extremities MM; IgG-κ 10% Multiple osteolytic lesions IV cyclophosphamide, bortezomib, dexamethasone; six cycles and autologous stem cell transplant Skin induration gradually decreased during treatment with complete recovery [12]
2015 62 (two patients)   Generalized symptomatic scleredema IgG MM    IV cyclophosphamide, bortezomib, dexamethasone, and IVIG Significant improvement [13]
  1. Abbreviations: IgG Immunoglobulin G, IV Intravenous, IVIG Intravenous immunoglobulin, MM Multiple myeloma, M male, F female