Table 1.
Summary of case features of localized cutaneous nodular amyloidosis in limited systemic sclerosis.
| Reference | Age, yrs | Sex | Length of LSSc Diagnosis (yrs) | Location of LCNA | yrs nodules present before presentation, progression | LCNA Treatments Tried, Results | LSSc Therapy | Negative systemic workup | Positive systemic workup | Follow up (yrs) |
|---|---|---|---|---|---|---|---|---|---|---|
| Summers and Kendrick, 2008 | 61 | Female | 1 | left shin | unknown, increasing in number and extending to bilateral lower extremities over the next 3 years | pulsed dye laser every 6 weeks, improved tenderness of lesions | unknown | CBC, glucose, serum-free light chains, paraproteinemia, SPEP/UPEP, RFT, echo, chest CT, skeletal survey, bone marrow biopsy | elevated LFT secondary to fatty liver | 8 |
| Shiman et al. 2010 | 83 | Female | 0 | bilateral lower extremities | 25, increasing in size and number over this time | unknown | unknown | unknown | unknown | unknown |
| Damian et al. 2010 | 61 | Male | 10 | bilateral lower extremities | 1.5 | patient declined therapy | unknown | CBC, serum free light chains, serum immunoelectrophroretogram, SPEP/UPEP, UA, LFT, RFT, TFT, echo, chest CT, abdomen CT, pelvis CT, abdominal biopsy, rectal biopsy | none | unknown |
| Current study | 71 | Female | 4 | left shin | 1 | monthly intralesional triamcinolone (10 mg/ml) injections over 3 months, modest improvement | omeprazole for esophageal dysmotility | CBC, BMP, SPEP/UPEP, PFT, echo | none | 1 |
| Current study | 58 | Female | 4 | bilateral lower extremities | 1.5 | monthly intralesional triamcinolone (10 mg/ml) injections over 4 months, modest improvement | unknown | echo, bone marrow biopsy | serum immunofixation electrophoresis, lung nodules of nodular amyloidosis | 9, diagnosed with Sjögren Syndrome at year 5 |
| Current study | 70 | Female | 22 | right shin | 4-5, enlarging over this time | surgically debulked, resolution and improved tenderness | amlodipine for Raynaud phenomenon, omeprazole for esophageal dysmotility, physical therapy for sclerodactyly | BMP, SPEP/UPEP, PFT, echo | leukopenia, subclinical hypothyroidism | 1 |
BMP: basic metabolic panel; CBC: complete blood count; CT: computed tomography; LCNA: localized cutaneous nodular amyloidosis; LFT: liver function tests; LSSc: limited systemic sclerosis; PFT: pulmonary function tests; RFT: renal function tests; SPEP/UPEP: serum protein electrophoresis/urine protein electrophoresis; TFT: thyroid function tests; echo: echocardiogram; UA: urinalysis.