Introduction
Case presentation
Investigation | Value | Normal range | Comment |
---|---|---|---|
WBC | 9.32 × 103/μl | 4–10 | Normal |
Lymphocytes | 2.17 × 103/μl | 0.8–4 | Normal |
Serum creatinine | 0.9 mg/dl | 60–120 | Normal |
Serum potassium | 3.4 mmol/L | 3.5–5.1 | Normal |
AST | 27 U/L | 10–35 | Normal |
Albumin | 38 g/L | 35–45 | |
Alkaline phosphatase | 104 U/L | 100–360 | Normal |
Ionized calcium | 1.21 mmol/ L | 1.0–1.3 | Normal |
Amylase | 68 U/ L | 22–80 | Normal |
Neutrophils | 6.09 × 103/μl | 2–7 | Normal |
Platelets | 277 × 103/μl | 150–450 | Normal |
Serum sodium | 138 mmol/L | 135–148 | Normal |
Normal | |||
ALT | 20 U/L | 10–40 | Normal |
INR | 1.26 | Normal | |
Serum magnesium | 1.7 mg/dl | 1.7–2.7 | Normal |
Troponin I | < 0.1 ng/ml | < 0.5 | Normal |
Discussion
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] |