Clinical Study

Lower Extremity Ulcers in Systemic Sclerosis: Features and Response to Therapy

Table 1

Features and outcomes of patients with scleroderma associated leg ulcers.

Pt12345678910

SexMMFFMFFFFF

RaceCCCCCHCCCC

SSc clinical subtypeSineLimitedLimitedDiffuseLimitedLimitedDiffuseLimitedLimitedSine

Disease duration at time of ulcer development (years)NA20174.144642624NA

Scleroderma-specific antibodyU3RNPCentromereCentromereRNA Pol3CentromereScl70CentromereScl70Centromere

Other AntibodiesNucleolar ANA; RFSpeckled ANASpeckled ANASSANucleolar ANASpeckled ANASpeckled ANA

Other scleroderma featuresGI dysmotility, GERDGI dysmotility, GERD, SICCA, limited skinGI dysmotility, SICCA, limited skin, joint contractures.Diffuse skin, SICCA, arthritis, interstitial lung disease, GI dysmotilitySICCA, limited skin, joint contracturesGERD, TelangiectasiasDiffuse skin, interstitial lung disease, GI dysmotility, SICCA, calcinosisLimited skin, joint contractures, SICCA, GERD, GI dysmotility, calcinosisLimited skin, arthritis, pulmonary hypertension, GERD, SICCA, GI dysmotilityCalcinosis

Ulcer locationLeft legLeft medial malleolusBilateral malleoli and right posterior ankleBilateral malleoliBilateral Toes, dorsal foot and heelBilateral lateral calfRight medial malleolus, left dorsal footBilateral malleoliLeft lateral malleolusBilateral toes and bottom of feet

Venous insufficiency on doppler US+

Arterial Disease on ABPI++

SCREEN 1LAC+++

β-2GPI (normal <10 U/mL)IgG39 10 10 10 10 1041 10 10 10
IgA 1013 10 10 1010023 10 10 10
IgM 10 10 10 10 10 10 10 10 10 10

ACL (normal <10 U/mL)IgG 10 10 1045 10 1023 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

SCREEN 2LAC+++NT
β-2GPI (normal <10 U/mL)IgG23 10 1019 10 10 10 10 10NT
IgA 1020 10 10 10100 10 10 10NT
IgM 10 10 10 10 10 10 10 10 10NT
ACL (normal <10 U/mL)IgG 10 10 1020 10 10 10 10 10NT
IgA 10 10 1014 10 10 10 10 10NT
IgM 10 10 10 10 10 10 10 10 10NT

Summary APLprofile+++++

Genetic procoagulant profileMTHFR012111011NT
PAI-1011020100NT
Prothrombin Gene000000000NT
FVL000000000NT

Summary Genetic procoagulant profile+++++++NT

BiopsyFibrin occlusive vasculopathyNo biopsyFibrin occlusive vasculopathyFibrin occlusive vasculopathyNo biopsyNo biopsyFibrin occlusive vasculopathyFibrin occlusive vasculopathyNo biopsyNo biopsy

TreatmentEnoxaparin 40 mg daily, arterioplastyArterioplastyEnoxaparin 1 mg/Kg twice dailyEnoxaparin stopped due to bleeding Darbepoetin alfaPentoxifylline 400 mg three times per dayEnoxaparin 40 mg dailyEnoxaparin 40 mg dailyNoneVenous surgery pendingHealed with Nifedipine

OutcomeHealedHealedNot healedHealedHealedHealed50% healing in 3 monthsNot healedNot healedHealed

Total duration of ulcer (months)3663501062336356

Time to healing after initiation of therapy463643

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.