Table 1.
Features and outcomes of patients with scleroderma associated leg ulcers.
| Pt | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | ||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Sex | M | M | F | F | M | F | F | F | F | F | ||
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| Race | C | C | C | C | C | H | C | C | C | C | ||
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| SSc clinical subtype | Sine | Limited | Limited | Diffuse | Limited | Limited | Diffuse | Limited | Limited | Sine | ||
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| Disease duration at time of ulcer development (years) | NA | 20 | 17 | 4.1 | 4 | 46 | 4 | 26 | 24 | NA | ||
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| Scleroderma-specific antibody | U3RNP | Centromere | Centromere | RNA Pol3 | Centromere | Scl70 | Centromere | Scl70 | Centromere | |||
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| Other Antibodies | Nucleolar ANA; RF | Speckled ANA | Speckled ANA | SSA | Nucleolar ANA | Speckled ANA | Speckled ANA | |||||
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| Other scleroderma features | GI dysmotility, GERD | GI dysmotility, GERD, SICCA, limited skin | GI dysmotility, SICCA, limited skin, joint contractures. | Diffuse skin, SICCA, arthritis, interstitial lung disease, GI dysmotility | SICCA, limited skin, joint contractures | GERD, Telangiectasias | Diffuse skin, interstitial lung disease, GI dysmotility, SICCA, calcinosis | Limited skin, joint contractures, SICCA, GERD, GI dysmotility, calcinosis | Limited skin, arthritis, pulmonary hypertension, GERD, SICCA, GI dysmotility | Calcinosis | ||
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| Ulcer location | Left leg | Left medial malleolus | Bilateral malleoli and right posterior ankle | Bilateral malleoli | Bilateral Toes, dorsal foot and heel | Bilateral lateral calf | Right medial malleolus, left dorsal foot | Bilateral malleoli | Left lateral malleolus | Bilateral toes and bottom of feet | ||
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| Venous insufficiency on doppler US | − | − | − | − | − | − | − | − | + | − | ||
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| Arterial Disease on ABPI | + | + | − | − | − | − | − | − | − | − | ||
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| SCREEN 1 | LAC | + | − | − | + | − | − | + | − | − | − | |
| β-2GPI (normal <10 U/mL) | IgG | 39 | <10 | <10 | <10 | <10 | <10 | 41 | <10 | <10 | <10 | |
| IgA | <10 | 13 | <10 | <10 | <10 | 100 | 23 | <10 | <10 | <10 | ||
| IgM | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | ||
| ACL (normal <10 U/mL) | IgG | <10 | <10 | <10 | 45 | <10 | <10 | 23 | <10 | <10 | <10 | |
| IgA | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | ||
| IgM | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | ||
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| SCREEN 2 | LAC | + | − | − | + | − | − | + | − | − | NT | |
| β-2GPI (normal <10 U/mL) | IgG | 23 | <10 | <10 | 19 | <10 | <10 | <10 | <10 | <10 | NT | |
| IgA | <10 | 20 | <10 | <10 | <10 | 100 | <10 | <10 | <10 | NT | ||
| IgM | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | NT | ||
| ACL (normal <10 U/mL) | IgG | <10 | <10 | <10 | 20 | <10 | <10 | <10 | <10 | <10 | NT | |
| IgA | <10 | <10 | <10 | 14 | <10 | <10 | <10 | <10 | <10 | NT | ||
| IgM | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | <10 | NT | ||
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| Summary APLprofile | + | + | − | + | − | + | + | − | − | − | ||
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| Genetic procoagulant profile | MTHFR | 0 | 1 | 2 | 1 | 1 | 1 | 0 | 1 | 1 | NT | |
| PAI-1 | 0 | 1 | 1 | 0 | 2 | 0 | 1 | 0 | 0 | NT | ||
| Prothrombin Gene | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | NT | ||
| FVL | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | NT | ||
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| Summary Genetic procoagulant profile | − | + | + | + | − | + | + | + | + | NT | ||
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| Biopsy | Fibrin occlusive vasculopathy | No biopsy | Fibrin occlusive vasculopathy | Fibrin occlusive vasculopathy | No biopsy | No biopsy | Fibrin occlusive vasculopathy | Fibrin occlusive vasculopathy | No biopsy | No biopsy | ||
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| Treatment | Enoxaparin 40 mg daily, arterioplasty | Arterioplasty | Enoxaparin 1 mg/Kg twice daily | Enoxaparin stopped due to bleeding Darbepoetin alfa | Pentoxifylline 400 mg three times per day | Enoxaparin 40 mg daily | Enoxaparin 40 mg daily | None | Venous surgery pending | Healed with Nifedipine | ||
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| Outcome | Healed | Healed | Not healed | Healed | Healed | Healed | 50% healing in 3 months | Not healed | Not healed | Healed | ||
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| Total duration of ulcer (months) | 36 | 6 | 350 | 10 | 6 | 23 | 36 | 3 | 5 | 6 | ||
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| Time to healing after initiation of therapy | 4 | 6 | — | 3 | 6 | 4 | — | — | — | 3 | ||
GERD: Gastroesophageal reflux disease; SICCA: dryness of the conjunctiva and cornea and dryness of the mouth; GI dysmotility: gastrointestinal dysmotility; ACL: anticardiolipin antibodies; β-2GP1 Ab: Beta-2 Glycoprotein I antibodies; LAC: lupus anticoagulant; MTHFR: Methyltetrahydrofolate reductase mutation; PAI-1: Plasminogen Activator Inhibitor-I mutation; FVL: Factor V Leiden mutation;
For gene mutation results 1: heterozygous mutation, 2: homozygous mutation; NT: not tested.