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convalescentphase, patientswith Charcotarthropathymay actuallynotedecreased swelling
and warmth, although damage that has already occurred does not reverse itself, and can
progress with continued weight-bearing activities. In the third reconstructive phase, healing
begins,withfracturedbonesanddeformedjointsbecomingsomewhatmorestable.Residual
instability may persist, however, and deformities of the foot that developed in the earlier
phasesmayincreasethelikelihoodofdevelopingulcerativeplantarlesions.
27
Tests
Radiographyisusefulforassessingthebonydestructionandpotentialjointcompromiseseen
with Charcot arthropathy. Plain films can also be used to stage disease and to monitor
progression(Fig.19-5).
Bonescansmay helpdifferentiateneuropathic jointarthropathyfrom osteomyelitis;MRI
imaging can provide greater anatomic detail and may also help differentiate Charcot
arthropathyfromosteomyelitis.
LaboratorytestshavelimitedutilityinevaluatingCharcotarthropathy,althoughnonspecific
markers of inflammation such as a complete blood count with differential, an erythrocyte
sedimentationrate,oraC-reactiveproteinmayindicatethatinfectionismorelikelythanpure
inflammationwhenelevated.Severalothertestsmayhelpconfirmorexcludethepresenceof
diabetes, including glucose levels and glycosylated hemoglobin, as many other causes of
neuropathicjointdiseaseareknown.Inmostcases,diabeticswhodevelopCharcotarthropathy
havehadperipheralneuropathyfor10to15years.
28
TreatmentsandTherapies
TheearlytreatmentofCharcotarthropathyaffectingthelowerextremitiesisaimedatarresting
progression of bony destruction and deformity. Serial contact casting using heavily padded
plasterorothercastingmaterialsmaycontrolswelling,andlimitprogressivedamage.Custommadewalkingbootsmayserveasimilarpurpose.Limitedweight-bearingmayalsoplayarole
in restricting damage. Bisphosphonate therapy and calcitonin are currently debated as
potentiallyusefuladjunctsinthemedicaltreatmentofCharcotarthropathy.
29
Surgicaltreatmentmayberequiredtostabilizeunstablejointsortorepairfracturedbones.
Amputation may be considered in cases where the feet are too damaged to allow weightbearing,inwhichcaseprostheticdevicesmaybeutilized.Patientsshouldalsobeeducatedon
how to protect the unaffectedor lesser affected foot as itis more heavily loaded when the
affectedfootismadenonweight-bearing.
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FIGURE19-5.RadiographsofclassicCharcotarthropathyshowingbonyprojections,fractures,dislocations,bone
disintegration,newboneformation,increaseddiastasisbetweenmetatarsalbases,andgrossalterationinskeletal
anatomy.(FromThordarsonD.OrthopaedicSurgeryEssentials:FootandAnkle.Philadelphia,PA:WoltersKluwer;
2012,withpermission.)
Specialized footwear and customized orthotics are often utilized in the long-term
management of Charcot arthropathy. Patients should be closely followed to monitor for
recurrentorworseningarthropathysymptoms,andtoensurethatorthoticscontinuetofitwell.
Lastly, diabetic patients must also be followed closely by their primary physician and
endocrinologisttooptimizelong-termglycemiccontrolanddiabeticdiseasemanagement.
SUMMARY
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Several systemic diseases may manifest wholly, or in part, with lower extremity findings.
Recognitionofthesignsandsymptomsinvolvingthelowerextremitiesthatportend systemic
illnessesoftenrequiresahighindexofsuspicion.Treatingtheprimarydiseaseprocessesthat
underlie the manifestations affecting the lower extremities is often necessary in order to
adequatelyaddress thesymptomatology that prompted a given patient toseekcare. In those
caseswherepatientsareunawareofayetundiagnosedsystemicillness,educationisnecessary
toensurethatthepatientappreciateshowmanagementoftheprimarydiseasecanimprovethe
overall outcome. Providing appropriate care beyond the initial presentation for acute
symptomatologytypicallyinvolvesaconcertedeffortonthepartofmanyproviders,aswellas
collaborationfromthepatientreceivingcare,tomaximizetheoveralloutcome.
REFERENCES
FeringaHH,BaxJJ,vanWaningVH,etal.Thelong-termprognosticvalueoftherestingandpostexerciseankle-brachial
index.ArchInternMed.2006;166(5):529–535.
KesselDO,BerridgeDC,RobertsonI.Infusiontechniquesforperipheralarterialthrombolysis.CochraneDatabaseSyst
Rev.2004;(1):CD000985.
BerridgeDC,KesselD,RobertsonI.Surgeryversusthrombolysisforacutelimbischaemia:initialmanagement.Cochrane
DatabaseSystRev.2013;(6):CD002784.
BartonA,WorthingtonJ.Geneticsusceptibilitytorheumatoidarthritis:anemergingpicture.ArthritisRheum.2009;61(10):
1441–1446.
ScottIC,Steers,LewisCM,etal.Precipitatingandperpetuatingfactorsofrheumatoidarthritisimmunopathology—linking
the triad of genetic predisposition, environmentalrisk factors and autoimmunity to disease pathogenesis. Clin Rheum.
2011;25(4):447–468.
KapitanyA,ZilahiE,SzantoS,etal.AssociationofrheumatoidarthritiswithHLA-DR1andHLA-DR4inHungary.Ann
NYAcadSci.2005;1051:263–270.
Van der Hejde DM. Radiographic imaging: the ‘gold standard’ for assessment of disease progression in rheumatoid
arthritis.Rheumatology.2000;39(1):9–16.
Wells AF,HaddadRH. Emergingroleofultrasonographyinrheumatoidarthritis: optimizingdiagnosis,measuringdisease
activityandidentifyingprognosticfactors.UltrasoundMedBiol.2011;37(8):1173–1184.
Faraj AA, Omonbude OD, Godwin P. Gram staining in the diagnosis of acute septic arthritis. Acta Orthop Belg.
2002;68(4):388–391.
HagenKB,ByfuglienMG,FalzonL,etal.Dietaryinterventionsforrheumatoidarthritis.Cochrane DatabaseSystRev.
2009;(1):CD006400.
SinghJA,FurstDE,BharatA,etal.2012updateofthe2008AmericanCollegeofRheumatologyrecommendationsfor
theuse of disease-modifying antirheumaticdrugsand biologic agents in thetreatmentof rheumatoid arthritis. Arthritis
CareRes.2012;64(5):625–639.
FellerL,KramerB,LemmerJ.Ashortaccountofmetastaticbonedisease.CancerCellInt.2011;11:24.
MaccauroG,SpinelliMS,MauroS,etal.Physiopathologyofspinemetastasis.IntJSurgOncol.2011;2011:107969.
Coleman RE. Metastatic bone disease: clinical features, pathophysiology and treatment strategies. Cancer Treat Rev.
2001;27:165–176.
BurgdorferW, BarbourAG,Hayes SF,et al.Lymedisease-atick-bornespirochetosis?Science. 1982;216(4552):1317–
1319.
Auwaerter PG. Point: antibiotic therapy is not the answer for patients with persisting symptoms attributable to lyme
disease.ClinInfectDis.2007;45(2):143–148.
GirschickHJ,MorbachH,TappeD.Treatmentoflymeborreliosis.ArthritisResTher.2009;11:258.
LymeDisease.http://www.cdc.gov/lyme/.UpdatedAugust2015.AccessedOctober22,2015.
NelsonC,HojvatS,JohnsonB,etal.Concernsregardinga new culturemethodforBorreliaburgdorferinotapproved
for the diagnosis of lyme disease. MMWR Morb Mortal Wkly Rep. 2014;63(15):333.
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6315a4.htm?s_cid=mm6315a4_w.AccessedJanuary17,2017.
WormserGP,DattwylerRJ,ShapiroED,etal.Theclinicalassessment,treatment,andpreventionoflymedisease,human
granulocyticanaplasmosis,andbabesiosis:clinicalpracticeguidelinesbytheInfectiousDiseasesSocietyofAmerica.Clin
https://t.me/medicina_free
21.
22.
23.
24.
25.
26.
27.
28.
29.
InfectDis.2006;43(9):1089–1134.
SmithBG,CruzAI, Milewski MD,et al.Lyme disease andthe orthopaedic implications oflymearthritis.J Am Acad
OrthopSurg.2011;19(2):91–100.
HirshJ,LeeAY.Howwediagnoseandtreatdeepveinthrombosis.Blood.2002;99(9):3102–3110.
KearonC.Naturalhistoryofvenousthromboembolism.Circulation.2003;107(23Suppl1):I22–I30.
FancherTL,White RH,KravitzRL.Combineduse ofrapidD-dimertestingandestimationofclinicalprobabilityinthe
diagnosisofdeepveinthrombosis:systematicreview.BMJ.2004;329:821–824.
Boulton AJ, Vinik AI, Arezzo JC, et al. Diabetic neuropathies: a statement by the American Diabetes Association.
DiabetesCare.2005;28(4):956–962.
RogersLC,FrykbergRG,ArmstrongDG,etal.Thecharcotfootindiabetes.DiabetesCare.2011;34(9):2123–2129.
vanderVenA,Chapman CB, BowkerJH. Charcotneuroarthropathyofthefootandankle.J AmAcad Orthop Surg.
2009;17(9):562–571.
Tan AL, Greenstein A, Jarret SJ, et al. Acute neuropathic joint disease: a medical emergency? Diabetes Care.
2005;28(12):2962–2964.
GüvenMF, KarabiberA,KaynakG, etal. Conservative and surgicaltreatment of the chronic Charcotfootandankle.
DiabetFootAnk le.2013;4:21177.
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BACKGROUND
Anoftenoverlookedcauseoflowerextremityulcersisdiseasesofthehematopoieticsystem.
Althoughulcersduetohematologicdiseasesarenotascommonasthoseduetovenous and
arterialdisorders,podiatricphysiciansstillneedtoincludesuchconditionsintheirdifferential
diagnosis. Ulcers of the foot and ankle secondary to blood dyscrasias may become more
commonasaresultofmodernmedicine’sabilitytokeeppatientsalivelongenoughtobecome
victimsofchronicdiseases.Also, manypharmaceuticalagentshaveamongtheirsideeffects
the ability to produce hematologic complications. The physician should not overlook
hematologic disorders as being among the etiologic agents responsible for foot and ankle
ulcers. Some ofthe conditionsin whichfootulcersmayoccurassociated with hematologic
disordersarediscussed.
SEVERITYOFFOOTANDLEGULCERS
Manyseverityindicesforfootandlegulcersarebasedonsize,depth,andduration.Staging
basedondepthisasfollows:
Stage1:Nonblanchableerythemaofintactskin,theheraldinglesionofskinulceration.In
individuals with darker skin, discoloration of the skin, warmth, edema, induration, or
hardnessmayalsobeindicators.
Stage2:Partial-thickness skin lossinvolving epidermis,dermis, or both. Theulceris
superficialandpresentsclinicallyasanabrasion,blister,orshallowcrater.
Stage3:Full-thicknessskinlossinvolvingdamagetoornecrosisofsubcutaneoustissue
thatmayextenddownto,butnotthrough,underlyingfascia.Theulcerpresentsclinically
asadeepcraterwithorwithoutunderminingofadjacenttissue.
Stage4:Full-thicknessskinlosswithextensivedestruction,tissuenecrosis,ordamageto
muscle,bone,orsupportingstructures(e.g.,tendon,jointcapsule).Underminingandsinus
tractsalsomaybepresent.
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SICKLECELLANEMIA
Sicklecellanemiaisanexampleofahematologicdiseasethatmaycauseulcersaffectingthe
lower extremities. TheU.S. Cooperative Study of Sickle Cell Disease (SCD) reported that
about 25% ofpatients with the disease over age 10 years hadahistoryof legulcers.1 The
diseaseaffects50,000to100,000peopleintheUnitedStates.2Itsgeographicaldistributionis
variable, affecting 75% of patients in Jamaica but only 8% to 10% of North American
patients.
3–5
Ulcersduetosicklecellanemiaarelesscommoninpatientswithα-genedeletion,
hightotalhemoglobinlevel,orhighlevelsofhemoglobinF.Thepathogenesisofchroniculcers
inSCDiscomplex.Mechanicalobstructionbydensesickledredcells,venousincompetence,
bacterialinfections,abnormalautonomiccontrolwithexcessivevasoconstrictionwheninthe
dependentposition,insituthrombosis,anemiawithdecreaseinoxygencarryingcapacity,and
decreasednitricoxide(NO)bioavailabilityleadingtoimpairedendothelialfunctionhaveall
beenproposed as potential contributing factors.
6,7
Patients havea markedreductionofNO,
whichisanimportantregulatorofvasculartone,celladhesion,andbloodflow.Thisresultsin
thedevelopmentofavasculopathyskewingthenormalbalancebetweenvasoconstrictionand
vasodilatation in favor of vasoconstriction. As a result, there is NO deficiency leading to
tissueischemiaandulcerformation.8Ulcersarepainfulandoftendisablingcomplicationsof
SCD,mayaffect5% to10% of adultpatients, andmaynecessitateopioids. Those thatare
acute usually heal within a month, butthose that are chronic may take 6 months.The SCD
ulcersaremorecommoninmalesandincreasewithage.Ulcersinsicklecellanemiatypically
occur in areas withthinskin and less subcutaneous fat, typically at the medial and lateral
malleoli. However, sometimes the dorsum of the foot is affected as is the region over the
Achillestendon.Theseulcersappearroundandpunched-outwithraisedmargins,deepbases
and necrotic slough varying in size from a few millimeters to large circumferential areas
coveringtheentiredistallowerextremity.Thelesionisusuallyprecipitatedbyminortrauma
and spreads in size gradually.
9,10
Sickle cell anemia ulcers frequently are colonized with
Staphylococcus aureus, Pseudomonas aeruginosa, and group A Streptococci. Typically
patientswiththeseulcersdevelopwoundinfectionswhichmaybelocalizedsuper-infections
orrecurrentcellulitisthathealslowlywithresidualhyperpigmentationandchronicscarring.
1
Healing of this difficulttotreatcondition is reported as being 20times slower thanin
similar types ofrefractoryvascular lesionsandis oftenrecurrent.11Preventionincludes the
reduction of trauma with properly fitted shoes and compression stockings supported by
applicationofemollientsalongwithproperhygiene.Whentheulcersoccurmoist,supportive
dressing are indicated, but when they drain, absorbent dressing could be used. Topical
antibioticssuchasneomycin,polymyxinB,orbacitracincanbeusedifsuperinfectionoccurs.
Whenthereisnecrotictissue,debridementisindicated.Therearereportsthatoralzincsulfate
accelerateshealing.
12,13
TheonlyFoodandDrugAdministration–approvedtreatmentofsickle
cellanemia–causedulcersinadultsishydroxyurea,acytotoxic,cytostaticdrugalsousedfor
thepreventionoftheulcers.Treatmentwithhydroxyureastartswithaninitialdoseof15mg
per kg (10 to 20 mg per kg) once a day.14 The dose may be increased in 5 mg/kg/day
incrementsevery12weekstoamaximumdoseof35mg/kg/day.However,otherssuggestthat
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hydroxyurea does not cause, prevent, or speed healing in SCD. Surgical intervention for
recalcitrant ulcers using split-thickness skingrafting andmyocutaneous flaps to bringblood
supplytotheareaofulcerationhasbeenused,butresultsareinconsistent.
ACUTELEUKEMIA
Inleukemia,specificornonspecificlesionsoftheskinmayoccur.Specificlesions(leukemia
cutis)containleukemiacells,whichdirectlyinfiltratetheepidermis,dermis,orsubcutaneous
fat.Nonspecificlesions,whicharemorecommon,areconsideredacutaneousreactiontothe
ongoingmalignancyprocess.
Therehavebeenreportsoffootulcersdevelopinginchildrenwithnewlydiagnosedacute
mixed lineage leukemia during induction chemotherapy. These ulcers may have a clinical
resemblance to pyoderma gangrenosum,buton histopathologic examination, herpes simplex
virusinfectionmaybediagnosed. Treatment withoral acycloviris ineffective,butthe ulcer
healedwithintravenous acyclovirfollowed byoral valacyclovir.Viral infectionremainsan
unusual but important cause of isolated extragenital cutaneous ulceration in the
immunocompromisedchild.
15
SPECIFICLESIONSINLEUKEMIACUTIS
Leukemia cutis includes granulocytic sarcoma, chloroma, and myeloblastoma, representing
localizedordisseminatedskininfiltrationbyblastcells.Leukemiacutisis usuallyasign of
relapseordissemination ofmedullarydisease.Itisuncommonbutismostlyfoundinpeople
older than 50 years. Most often, it is associated with acute monocytic leukemia and acute
myelomonocyticleukemia.Itcanbeobservedinanyacuteorchronicleukemia,includingthe
leukemicphaseofnon-Hodgkinlymphomaandhairycellleukemia.Skinpain,tenderness,and
pruritusareusuallyabsent,andmostcommonly,lesionsarediscrete,firm,violaceousorredbrownpapules,nodules,orplaques.Onoccasion,ulcerativelesionsareobserved.
Thedifferentialdiagnosesincludebacterial,viral,andfungaldermatoses;drugeruptions;
leukocytoclastic vasculitides; Sweetsyndrome; and pyoderma gangrenosum. Leukemia cutis
may occur simultaneously with or before the medullary disease. Leukemia cutis typically
develops late inthe course ofestablished acuteleukemia. Incontrast, when it develops in
associationwith chronic myelogenous leukemia or other myeloproliferative disorders, it is
usuallyasignofimpendingblastictransformation.Earlycutaneousinvolvementoccursmore
frequently in acute myelomonocytic leukemia and monocytic leukemia. In addition, it is
stronglyassociatedwithextramedullaryinvolvementofothersites,includingthemeninges.
Histopathologically, leukemia cutis includes a diffuse and infiltrative population of
mononuclearcellsaccompaniedbygranulocyticcellsatvariousstagesofmaturationtypically
includingeosinophilicmyelocytes.Criteriafordiagnosingleukemiacutisonskinbiopsyare:
chloroacetate esterase activity and Leu-M1 positivity; and the presence of lysozyme, α1-
antitrypsin, and α1-antichymotrypsin serve as supportive findings, with lysozyme being the
mostconsistentpositivemarker.
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The prognosis for leukemia cutis is related directly to the prognosis for the systemic
disease.
16
THALASSEMIAMAJORANDMINOR
Footulcersduetothalassemiaminorarerare,buttherehavebeencasesreported.A31-yearold Greek womanwith thalassemia minor developed multiple painful ulcers on the ankles,
toes,anddorsaofbothfeet,whichwerewelldefinedandmeasured2to10mmindiameter.
Alsoobservedwerepunctatepetechialhemorrhages.Anacutevasculitisofthecapillariesand
fibrinoid necrosis of the vessel walls were observed microscopically with petechial
hemorrhages and a lymphocytic infiltration in the surrounding connective tissue. No
abnormalitieswereseenwithlymphangiography,phlebography,andarteriographyofthelower
limbs. Although also rare, thalassemia major (Cooley anemia) ulcers have been reported.
Patientswiththalassemiamajorusuallyhavehemoglobinlevelsof9gorless,andulcersthat
formareprobablyduetolackofoxygenasaresultoflowhemoglobinA.Theseulcers,like
thalassemia minor, are usually found around the ankles unilaterally or bilaterally. Also a
possiblecauseoflegulcersis thalassemiaintermedia, whichoccursinpatientswithpoorly
controlledoruntreateddisease.Thismaybeduetopooroxygendeliverybecauseofthehigh
oxygenaffinityofhemoglobinFthatmaybemorethan50%ofthepatient’stotalhemoglobin.
17
THROMBOCYTHEMIAANDTHROMBOCYTOSIS
Inthrombocythemia,bloodclotsmaydevelopinthefeetaswellasthebrainandhands,less
commonly,inotherpartsofthebodysuchastheheartandintestines.Whentheyaffectthefeet,
theremaybetingling.
Thesignsandsymptomsofa highplateletcountarelinkedtobleedingandblood clots.
Symptomsmayalsoincludeulcersaffectingthetoes.Bloodclotsinthe smallvessels ofthe
feet resultinnumbness and redness. This may lead to intense burning and throbbinginthe
plantar aspectofthefoot.Bleeding occurs when theplatelet count is higher than 1 million
plateletspermicroliterofblood.
CRYOGLOBULINEMIA
Initially described by Wintrobe and Buell in 1933, cryoglobulins (CGs) were found in a
patient withmultiple myeloma.PatientswithCGs are classifiedintothreetypes.TypeIare
monoclonal, composed of immunoglobulin (Ig)M, IgG, IgA, and Bence Jones proteins in
decreasingorderoffrequency.TheserumlevelofmonoclonalCGsistypicallyhigh,andthey
precipitateeasilyatcoldtemperatures.TypeIICGsaremixedCGscomposedofcomplexesof
polyclonal IgG and monoclonal IgM rheumatoid factor.Type III CGs are mixed polyclonal
CGs,mostcommonlycomposedofIgMandIgGandareusuallypresentatverylowlevelsin
the serum.Type Icryoglobulinemiais associated withmultiple myeloma, other hematologic
proliferativedisorders,rheumatoidarthritis,andautoimmunehemolyticanemia.TypeIIandIII
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cryoglobulinemias are associated with chronic lymphocytic leukemia or lymphoma.
Autoimmunediseases,suchasSjögrensyndromeandrheumatoidarthritis,arefoundinpatients
with type II cryoglobulinemia. Type III cryoglobulinemia is seen in patients with systemic
lupus erythematosus (SLE), periarteritis nodosa, idiopathic thrombocytopenic purpura, and
hematologic proliferative disorders. Infections, including hepatitis B, mononucleosis,
cytomegalovirus,subacutebacterialendocarditis,andtoxoplasmosis,canbefoundinpatients
withcryoglobulinemias.Thetermessentialcryoglobulinemiadescribescryoglobulinemiawith
no clinical signs of underlying disease. Cryoglobulinemia was initially noted to occur
predominantlyinpatientswithmyeloma,butitisnowbeingdetectedinagrowingnumberof
infectious, collagen-vascular, and lymphoproliferative disorders. The major cause of
cryoglobulinemiaisnowconsideredtobehepatitisCvirus(HCV).Cutaneousmanifestations
are themost typical clinical sign of cryoglobulinemic vasculitis. They range from palpable
purpura of the lower limbs to chronic torpid cutaneous ulcers typically located in the
supramalleolarregions.Thecutaneousinvolvementisoftencomplicatedbytheoccurrenceof
chroniclegulcerswithlittleornotendencytohealandwhichspontaneouslycausepainand
severediscomforttothepatient.AcaseofHCV-positivetypeIcryoglobulinemiawithsevere
legulcersandnotresponsivetoantiviralandimmunosuppressivetreatmentisdescribed.This
was followed by double filtration plasmapheresis for 6 months, with no other associated
treatment. Before and after each session, an assessment of immunoglobulins, complement,
cryocrit, and fibrinogen was made. HCV RNA levels were determined in serum
cryoprecipitate,inthesupernatantbeforeandaftereachsession,andinthecollectionbag.No
differencesinpre- andpostapheresis valueswereobservedintheserum concentrationsand
the supernatant. However, the postapheresis cryoprecipitate showed a significantly reduced
viralload(P<0.02)ascomparedwiththepreapheresisvalues.Improvementintheulcersin
thelegoccurredduringapheresiswithcompleteregressionbytheendofthecycle.Skinbiopsy
specimens from patients with primary mixed IgM–IgG cryoglobulinemia were examined by
immunofluorescence,light,andelectronmicroscopy.Thebiopsiestakenfromtheinvolvedskin
of one patient with leg ulcers revealed small blood vessel occlusions by CG aggregates.
Because asimilarfinding wasnotobserved inthebiopsymaterialtakenfromtheother five
patientswhohadnoulcerativeskinlesions,itseemsthattheCGaggregatesplayaroleinthe
developmentoftheskinulcerationsinprimarymixedIgM–IgGcryoglobulinemia.
18–20
MYELOPROLIFERATIVEDISORDERS
Asaresultofimpairmentofthecutaneousmicrocirculation, hemorrheology,orthe effectof
hydroxyurea, ulcerations of the leg may occur in myeloproliferative disorders. The use of
hydroxyurea mayhaveasasideeffectthepresentationofsuchlegulcers.Infact,theuseof
hydroxyurea in legulcers mayresultin a wound size increase. Ifthe legulcers are dueto
hydroxyurea and the drug is stopped, typically healing occurs. Anoption is touse another
cytotoxic agent if one is available, but it too may result in a leg ulcer. The differential
diagnosis of myeloproliferative disease is vasculitis, cryoglobulinemeia, and pyoderma
gangrenosum.
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ANTIPHOSPHOLIPIDSYNDROME
Antiphospholipid syndrome (APS) is an acquired, multisystemic disorder characterized by
recurrentthrombosesinthearterialsystem,venoussystem,orboth.APSisclassifiedintotwo
groups:primaryandsecondary.
SecondaryAPSisoftenassociatedwithSLEandinfrequentlywithotherdiseases,suchas
lymphoproliferativedisorders,autoimmunediseases,infections(e.g.,syphilis,HIV,HCV),and
drugs (e.g., procainamide, quinidine, hydralazine, phenytoin, chlorpromazine). Serologic
markers for APSare antiphospholipid antibodies (β2-glycoprotein I or anticardiolipins) or
lupusanticoagulant.
Theprimarydiagnosticcriteriaincludearterial thrombosis, venous thrombosis, recurrent
fetalloss,andthrombocytopenia.Oneofthelistedprimarycriteriaisrequiredfordiagnosis,
combinedwithasustainedelevatedtiterofIgGanticardiolipinorlupusanticoagulant,which
canbedetectedbasedontheprolongedactivatedpartialthromboplastintime,Kaolinclotting
time,ordiluteRussellVipervenomtime.
A large retrospective study from the Mayo Clinic of patients with lupus anticoagulant
included41%whohadskinlesionsasthefirstsignofAPS.Cutaneousmanifestationsinclude
livedo reticularis, necrotizing vasculitis, livedo vasculitis, thrombophlebitis, cutaneous
ulcerationandnecrosis,erythematousmacules,purpura,ecchymosis,painfulskinnodules,and
subungualsplinterhemorrhages.
21,22
LIVEDORETICULARIS
Occasionally, livedo reticularis will cause ulcers of the foot. The disease is a condition
associated with reddish blue, mottled, reticular, or blotchy discoloration that resembles a
fishnet. Ulcerations occur onlyrarely, starting primarilyinthesummermonths.A case was
reported in which ulcers were present and caused by IgA anticardiolipin antibodies. As a
resultoftreatmentwithwarfarinandchloroquineaswellasgivingupbirthcontrolpillsand
smoking,cardiolipincanbeincludedinallcasesoflivedoreticularisbecausetreatmentmay
bemoreeffectiveintheeventofpositiveantibodydetection.
23
REFERENCES
Sehgal S, Arunkumar B. Microbila flora and its significance in pathology of sickle cell disease leg ulcers. Infection.
1992;20(2):86–88.
BrawleyO,CorneliusL,Edwards L, etal. NIH Conference Annals ofInternalMedicine NationalInstitutesof Health
Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. Ann Intern Med.
2008;148(12):932–938.
HerrickJB. Peculiar elongatedandsickle-shapedredblood corpusclesin a case of severe anemia. Yale J Biol Med.
2001;74(3):179–184.
CummerC,LaRoccoC.Ulcersofthelegsinsicklecellanemia.ArchDermatol.1940;52(6):1015–1039.
NolanV,AdewoyeA,BaldwinC,et al.Sickle cellulcers:associatedwithhaemolysisandSNPsssinKlotho,TEK and
genesoftheTGF-B/BMPpathway.BrJHaematol.2006;133(5):570–578.
TrentJ,KirsnerR.Legulcersinsicklecelldisease.AdvSkinWoundCare.2004;17(8):410–416.
WolfortF,KrizekT.Skinulcerationinsicklecellanemia.PlastReconstrSurg.1969;43(1):71–77.
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