Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2821_Библиотеки_им_академика_М_И_Перельмана
.pdf
adrenocorticotropic hormone (ACTH) secreted by the pituitary gland. Hypersecretion of
ACTH by a pituitary adenoma or, more rarely, via ectopic production of ACTH by a
nonpituitarycarcinomasuchassmallcelllungcancercanleadtoelevatedlevelsofcortisol.
7
Similarly,directproductionofcortisolbyanadrenalcorticaladenomaorcarcinomacanalso
rarelyleadtoelevatedcortisollevels.
The clinical manifestations of Cushing syndrome include central weight gain with a
characteristicroundmoonfaceandbuffalohump.8Thinningoftheskinwitheasybruising,as
well as reddish-purple striationson thetrunk andlegs,is also seen. Other features include
proximal muscle weakness, acne, hirsutism, hypertension, hyperglycemia, and bone loss.
Notably,incontrasttotheobservedtruncalweightgain,theextremitiesmaybecomequitethin
secondarytoassociatedmusclewasting,andslowhealingofcutsandbruisesmayalsooccur.
Matched cohort studies also underscore an elevated risk of thromboembolic disease and
infections.
9
ADDISONDISEASE
Addisondisease or primaryadrenocorticaldeficiencyisdefinedbyloss ofadrenalcortical
function, most commonly because of autoimmune destruction.10 The resultant loss of
glucocorticoidandmineralocorticoidproductionbytheadrenalcortexcanprecipitatealifethreatening adrenal crisis marked by hypotension and volume depletion.11 Many of the
preceding symptoms are subtle, and include fatigue, nausea, orthostasis, salt craving, and
muscleandjointpain.Notably,patientsmayalso manifestskinhyperpigmentation duetothe
factthatbothACTH,whichiselevatedinthesettingoflowglucocorticoids,andmelanocyte-
stimulating hormone share the same hormone precursor.12 Skin darkening occurs in sunexposedareas,butalsocharacteristicallyinpalmarcreases,recentscars,andpressurepoints
suchasthekneesandknuckles.
DIABETES
Diabetesisgenerallydivided intoinsulin-dependentandnon–insulin-dependentvarieties,or
TypeIandTypeII,respectively.TypeI,alsoknownasjuvenileonset,hasapeakincidencein
patients younger than age 14, but can also occur in adults.13 It is caused by selective
autoimmune destruction of the pancreas’ islets of Langerhans (beta cells), with resultant
inability to produce insulin.14 In contrast, Type II occurs most often in middle age and is
hallmarked by peripheral tissue resistance to insulin, particularly skeletal muscle but also
hepaticandadiposecells.15Inthelongrun,TypeIIdiabetescanalsoleadtolossofbetacells
andhyperglycemia.
Elevated serum glucose can lead to blood vessel atherosclerosis through a number of
mechanisms.Nonenzymaticglycosylationofcriticalproteinsandlipidsoccursinvesselwall
endothelial cells, smooth muscle cells, and macrophages, which can lead to their
dysfunction.
16,17
In turn, receptor activation by glycosylated proteins can lead to oxidative
https://t.me/medicina_free
stressandalterationsingrowthfactorexpression.Peripheralarterydiseaseisacontributing
componentofanestimated25%to30%ofdiabeticulcerations.
18
Distalsymmetricpolyneuropathyalsocharacterizesdiabetesandisasignificantsourceof
morbidity.Atleast50%ofdiabeticswilldevelopneuropathy,whichcanbemarkednotonly
by decreased sensation,but also by“positive” symptoms such as burningandtingling.19 Its
causesincludeacombinationofdirectnerve injury from hyperglycemiaand ischemicinsult
from microvascular dysfunction. The loss of protective sensation contributes to almost all
diabeticulcerations,andover60%ofulcerationsresultfromatriadofneuropathy,minorfoot
trauma,andfootdeformity.
18,20
Furthermore, decreased joint mobility occurs in both large and small joints due to
stiffening of collagen-containing tissues.
21,22
Decreased mobility of the ankle or 1st
metatarsophalangealjointdirectlycorrelateswiththeseverityofdiabeticneuropathy.Thisin
turnincreasesplantarpressure,whichcancontributetoulcerations.
Notably,asmallportionofpatientswhohavepoorlycontrolleddiabetesdevelopanacute
andpainful“insulinneuritis”uponinitiatinginsulinforglycemiccontrol.19Itismorecommon
amongTypeIdiabetics,butcanalsooccurinthosewithTypeII.Thepainisself-limitedin
durationbutcan be quite severe, andcan recur withsubsequent lapses in glycemic control
followedbyreinitiationofinsulintherapy.
THYROIDHORMONE
Thehormonesthyroxine(T4)andtriiodothyronine(T3)areproducedbythethyroidglandunder
anaxisofhypothalamicandpituitarycontrol.TargettissuesinturnconvertT4toT3,whichis
the morebiologically active form. Thesehormonesplay animportantrole inreaching adult
stature and act in concert with GH to promote bone formation.23 They also affect physeal
closure, wherein patients with juvenile thyrotoxicosis have accelerated growth and early
skeletaldevelopment,whereasthosewithhypothyroidismfrequentlyhaveaboneagethatlags
behindchronologicage.
24
Excess or a deficiency of thyroid hormone in adults can lead to osteoporosis and
fractures.
24,25
Studieshavefoundasignificantlyincreasedriskoffractureupondiagnosis of
hyperthyroidism (incidence rate ratio [IRR] between 1.26 and 2.29), with a concomitant
decreased riskafter surgical treatment (relative risk= 0.66, 95% confidence interval [CI]:
0.55to0.78)orinitiationofantithyroidmedication.
26,27
Similarly, anincreased riskis also
foundin patients with hypothyroidism (IRRbetween2.17and 2.35), but this riskabates 10
yearsafterdiagnosis.
Hypothyroidism may also increase the risk of wound dehiscence after surgical
procedures.28Amongpatientsundergoingfootandankleprocedures,athreefoldincreaseinthe
rateofwounddehiscencehasbeenobservedaftercorrectingforage,gender,hypertension,and
peripheral vascular disease (odds ratio =3.7; 95% CI: 1.3 to 11.4; P = 0.01).29 Proposed
mechanismsincludealterationsinkeratingeneexpressionandtheeffectofhypothyroidismon
collagentypepredominance.
30,31
https://t.me/medicina_free
PARATHYROIDHORMONE
Parathyroidhormone(PTH)isthemajorregulatorofserumcalcium,synthesizedbythechief
cellsoftheparathyroidglands.32AdecreaseinserumcalciumstimulatesthesecretionofPTH
through a negative feedbackloop. In turn,PTH increases serum calcium by triggering bone
resorption, increased renal tubule absorption of calcium, and an increase in intestinal
absorptionofcalciumviaPTH’seffectsonvitaminD.
The most common cause of hyperparathyroidism is because of chronic renal failure,
wherebyadecreased abilityof the kidneystosecrete phosphateresultsinionic bindingof
serum calcium.33 Furthermore, renal activation of vitamin D is also impaired, leading to
decreased intestinal absorption of calcium. The resultant decrease in serum calcium
concentrationsstimulatesPTHsecretion.Primaryhyperparathyroidismcanalsobecausedbya
parathyroid adenoma, which results in oversecretion of the hormone independent of serum
calciumconcentrations.
34
Regardless of itscause,hyperparathyroidism stimulates osteoclastic resorption ofbone,
causingcysticchangestothebone.Whensevere,fibrousreplacementofthesecysticareasmay
leadtonon-neoplastic,tumor-likemassesknownasbrowntumors,namedforthecoloroftheir
hemosiderindeposits.
35,36
Pathologicfracturesthroughbrowntumorshavebeendescribed,and
treatmentultimatelyincludesamelioratingtheunderlyingcauseofhyperparathyroidism.
37
Hyperparathyroidismcanalsobeassociatedwiththedepositionofcalciumpyrophosphate
crystalsinjointsandothersofttissues,knownaspseudogout.38Althoughthevastmajorityof
patients with pseudogout do not have hyperparathyroidism, perhaps 20% of patients with
hyperparathyroidism develop pyrophosphate arthropathy.39 Interestingly, surgical
parathyroidectomy can itself precipitate pseudogout attacks.40 Furthermore, over half of
patients with pseudogout in the knee will also demonstrate ultrasonographic features of
pyrophosphatecrystalsintheAchillestendonandaquarterintheplantarfascia.
41
Interestingly, although chronically high levels of PTH can lead to bone resorption,
intermittent,lowdosesofPTHareanabolicinnature,resultinginan increasedbonedensity
anddecreasedrateofosteoporoticfractures.42Thismaybepartlythroughadirecteffecton
osteoblast lineage cells, but also through indirect effects on skeletal growth factors. PTH
therapieshavethereforeincreasinglybecomeamainstayofosteoporoticmanagement.
Hypoparathyroidism is most commonly caused by accidental removal during surgical
excisionofthethyroidgland.43Hypocalcemiamayinturn leadtoneuromuscular excitability
and,inseverecases,tetany.
44
SUMMARY
Inadditiontotheirdiffuseeffectsacrossnumerousorgansystems,hormonesfrequentlydirectly
impactnormalmusculoskeletalfunction.Derangementsintheendocrinesystemoftenunveilthe
delicateequilibriumunderwhichhormonelevelsmustbemaintained.
https://t.me/medicina_free
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
23.
24.
REFERENCES
MeinhardtU,NelsonAE,HansenJL,etal.Theeffectsofgrowthhormoneonbodycompositionandphysicalperformance
in recreational athletes: a randomized trial. Ann Intern Med. 2010;152(9):568–577. doi:10.7326/0003-4819-152-9-
201005040-00007.
Ezzat S, Asa SL, Couldwell WT, et al. The prevalence of pituitary adenomas: a systematic review. Cancer.
2004;101(3):613–619.doi:10.1002/cncr.20412.
ReidTJ,PostKD,Bruce JN,etal.Features atdiagnosisof324patientswithacromegalydid notchangefrom1981to
2006: acromegaly remains under-recognized and under-diagnosed. Clin Endocrinol (Oxf). 2010;72(2):203–208.
doi:10.1111/j.1365-2265.2009.03626.x.
Mankin HJ, Jupiter J, Trahan CA. Hand and foot abnormalities associated with genetic diseases. Hand (NY).
2011;6(1):18–26.doi:10.1007/s11552-010-9302-8.
Carroll TB, Findling JW. Cushing’s syndrome of nonpituitary causes. Curr Opin Endocrinol Diabetes Obes.
2009;16(4):308–315.doi:10.1097/MED.0b013e32832d8950.
Prague JK, May S, Whitelaw BC. Cushing’s syndrome. BMJ. 2013;346:f945.
http://www.ncbi.nlm.nih.gov/pubmed/23535464.AccessedSeptember14,2014.
MazzonePJ,ArroligaAC.Endocrineparaneoplasticsyndromesinlungcancer.CurrOpinPulmMed.2003;9(4):313–320.
http://www.ncbi.nlm.nih.gov/pubmed/12806246.AccessedSeptember14,2014.
Kirk LF, Hash RB, Katner HP, et al. Cushing’s disease: clinical manifestations and diagnostic evaluation. Am Fam
Physician. 2000;62(5):1119–1127, 1133–1134. http://www.ncbi.nlm.nih.gov/pubmed/10997535. Accessed September 14,
2014.
DekkersOM,Horváth-PuhóE,JørgensenJOL,etal.MultisystemmorbidityandmortalityinCushing’ssyndrome:acohort
study.JClinEndocrinolMetab.2013;98(6):2277–2284.doi:10.1210/jc.2012-3582.
MichelsA,MichelsN.Addisondisease:earlydetectionandtreatmentprinciples.AmFamPhysician.2014;89(7):563–568.
http://www.ncbi.nlm.nih.gov/pubmed/24695602.AccessedSeptember14,2014.
Chakera AJ, Vaidya B. Addison disease in adults: diagnosis and management. Am J Med. 2010;123(5):409–413.
doi:10.1016/j.amjmed.2009.12.017.
Pritchard LE, Turnbull A V, White A. Pro-opiomelanocortin processing in the hypothalamus: impact on melanocortin
signalling and obesity. J Endocrinol. 2002;172(3):411–421. http://www.ncbi.nlm.nih.gov/pubmed/11874690. Accessed
September14,2014.
Dabelea D, Bell RA, D’Agostino RB, et al. Incidence of diabetes in youth in the United States. JAMA.
2007;297(24):2716–2724.doi:10.1001/jama.297.24.2716.
WillcoxA,RichardsonSJ,BoneAJ,etal. Analysisofislet inflammationin humantype 1diabetes.ClinExp Immunol.
2009;155(2):173–181.doi:10.1111/j.1365-2249.2008.03860.x.
DeFronzo RA,Tripathy D. Skeletal muscle insulin resistance isthe primarydefect in type 2diabetes. Diabetes Care.
2009;32(suppl2):S157–S163.doi:10.2337/dc09-S302.
Aronson D. Hyperglycemia and the pathobiology of diabetic complications. Adv Cardiol. 2008;45:1–16.
doi:10.1159/0000115118.
Chait A, Bornfeldt KE. Diabetes and atherosclerosis: is there a role for hyperglycemia? J Lipid Res.
2009;50(Suppl):S335–S339.doi:10.1194/jlr.R800059-JLR200.
WuSC,Driver VR, Wrobel JS, et al.Footulcers inthe diabetic patient, preventionand treatment. Vasc Health Risk
Manag. 2007;3(1):65–76. http://www.pubmedcentral.nih.gov/articlerender.fcgi?
artid=1994045&tool=pmcentrez&rendertype=abstract.AccessedSeptember16,2014.
Smith AG, Singleton JR. Diabetic neuropathy. Continuum (Minneap Minn). 2012;18(1):60–84.
doi:10.1212/01.CON.0000411568.34085.3e.
ReiberGE,VileikyteL,BoykoEJ,etal.Causalpathwaysforincidentlower-extremityulcersinpatientswithdiabetesfrom
twosettings. Diabetes Care.1999;22(1):157–162. http://www.ncbi.nlm.nih.gov/pubmed/10333919. Accessed September
16,2014.
Singh N, Armstrong DG, Lipsky BA. Preventing foot ulcers in patients with diabetes. JAMA. 2005;293(2):217–228.
doi:10.1001/jama.293.2.217.
ZimnyS,SchatzH,Pfohl M.The role oflimited jointmobilityin diabeticpatients with anat-risk foot.Diabetes Care.
2004;27(4):942–946.http://www.ncbi.nlm.nih.gov/pubmed/15047653.AccessedSeptember16,2014.
Nilsson O, Marino R, De Luca F, et al. Endocrine regulation of the growth plate. Horm Res. 2005;64(4):157–165.
doi:10.1159/000088791.
Bassett JHD,WilliamsGR. Critical role of the hypothalamic-pituitary-thyroid axis in bone. Bone. 2008;43(3):418–426.
https://t.me/medicina_free
25.
26.
27.
28.
29.
30.
31.
32.
33.
34.
35.
36.
37.
38.
39.
40.
41.
42.
43.
44.
doi:10.1016/j.bone.2008.05.007.
WirthCD,Blum MR, daCostaBR,etal.Subclinicalthyroiddysfunctionandtheriskforfractures:a systematic review
andmeta-analysis.AnnInternMed.2014;161(3):189–199.doi:10.7326/M14-0125.
VestergaardP,MosekildeL.Fracturesinpatientswithhyperthyroidismandhypothyroidism:anationwidefollow-upstudy
in16,249patients.Thyroid.2002;12(5):411–419.doi:10.1089/105072502760043503.
Vestergaard P, Rejnmark L, Mosekilde L. Influence of hyper- and hypothyroidism, and the effects of treatment with
antithyroiddrugsandlevothyroxineonfracturerisk.CalcifTissueInt.2005;77(3):139–144.doi:10.1007/s00223-005-0068-
x.
ShermakMA,ChangD,MagnusonTH,etal.Anoutcomesanalysisofpatientsundergoingbodycontouringsurgeryafter
massiveweightloss.PlastReconstrSurg.2006;118(4):1026–1031.doi:10.1097/01.prs.0000232417.05081.db.
Grunfeld R,KunselmanA,Bustillo J,et al. Wound complications inthyroxine-supplementedpatients followingfoot and
anklesurgery.FootAnkleInt.2011;32(1):38–46.doi:10.3113/FAI.2011.0038.
Safer JD, Crawford TM, Holick MF. A role for thyroid hormone in wound healing through keratin gene expression.
Endocrinology.2004;145(5):2357–2361.doi:10.1210/en.2003-1696.
TháNassifAC,HintzGreca F,GrafH,etal. Wound healingin colonic anastomosis in hypothyroidism.Eur Surg Res.
2009;42(4):209–215.doi:10.1159/000208519.
Lee M, Partridge NC. Parathyroid hormone signaling in bone and kidney. Curr Opin Nephrol Hypertens.
2009;18(4):298–302.doi:10.1097/MNH.0b013e32832c2264.
GoodmanWG.Medicalmanagementofsecondaryhyperparathyroidisminchronicrenalfailure.NephrolDialTransplant.
2003;18(Suppl3):iii2–iii8.http://www.ncbi.nlm.nih.gov/pubmed/12771290.AccessedMarch31,2015.
WienekeJA,SmithA.Parathyroidadenoma.HeadNeckPathol.2008;2(4):305–308.doi:10.1007/s12105-008-0088-8.
Nassar GM, Ayus JC. Images in clinical medicine. Brown tumor in end-stage renal disease. N Engl J Med.
1999;341(22):1652.doi:10.1056/NEJM199911253412204.
QuinnCE,HealyJ,Lebastchi AH,etal. Modernexperiencewithaggressiveparathyroidtumorsinahigh-volumeNew
Englandreferralcenter.JAmCollSurg.2014.doi:10.1016/j.jamcollsurg.2014.10.007.
Vernace N, Bancroft LW. Multiple brown tumors and pathologic patellar fracture in a patient with secondary
hyperparathyroidism.Orthopedics.2014;37(8):564–567.doi:10.3928/01477447-20140728-01.
RichetteP,BardinT, DohertyM. Anupdate ontheepidemiologyof calciumpyrophosphate dihydrate crystaldeposition
disease.Rheumatology(Oxford).2009;48(7):711–715.doi:10.1093/rheumatology/kep081.
HuauxJP, GeubelA,KochMC,etal.Thearthritisofhemochromatosis.Areviewof25cases withspecial referenceto
chondrocalcinosis, and a comparison with patients with primary hyperparathyroidism and controls. Clin Rheumatol.
1986;5(3):317–324.http://www.ncbi.nlm.nih.gov/pubmed/3490947.AccessedMarch31,2015.
Rubin MR, Silverberg SJ. Rheumatic manifestations ofprimary hyperparathyroidismand parathyroid hormone therapy.
CurrRheumatolRep.2002;4(2):179–185.http://www.ncbi.nlm.nih.gov/pubmed/11890884.AccessedMarch31,2015.
Filippou G, Filippucci E, Tardella M, et al. Extent and distribution of CPP deposits in patients affected by calcium
pyrophosphate dihydrate deposition disease: an ultrasonographic study. Ann Rheum Dis. 2013;72(11):1836–1839.
doi:10.1136/annrheumdis-2012-202748.
CanalisE,GiustinaA,BilezikianJP.Mechanismsofanabolictherapiesforosteoporosis.NEnglJMed.2007;357(9):905–
916.doi:10.1056/NEJMra067395.
LinDT,PatelSG,ShahaAR,etal.Incidenceofinadvertentparathyroidremovalduringthyroidectomy.Laryngoscope.
2002;112(4):608–611.doi:10.1097/00005537-200204000-00003.
DeSanctisV,SolimanA,FiscinaB.Hypoparathyroidism:fromdiagnosistotreatment.Curr Opin EndocrinolDiabetes
Obes.2012;19(6):435–442.doi:10.1097/MED.0b013e3283591502.
https://t.me/medicina_free
A
s the body’s point of contact with the weight-bearing surface, the foot is constantly
bombardedwithdermatologicinsults.Itresistsabrasiveforcesforalifetime,yetrequires
littlecareinreturn.Itisthesiteofawidevarietyoflocalandsystemicafflictions,someeven
portending a state of relative emergency. This chapter hopes to present an algorithmic
approachusefulinaclinicalsetting,payingcarefulattentiontotheconsiderationandexclusion
ofseriousorsystemicconditions.
DERMATOLOGICSYMPTOMSANDSIGNS
Theprimarydermatologicsymptom is pruritus,anditsdiscoveryhelpstosupportthenotion
thatadermatologicchangeisindeedtheresultofskindiseaseasopposedtoalterationcaused
by affliction of another organ system. This is best illustrated by the scaling and
hyperpigmentation observed approximately 2 weeks after acute gouty arthritis. On initial
examination,thesefindingsmightsuggesttineapedisorotherprimaryskindisease;however,
thechangesareinfacttheresultofarheumatologicratherthandermatologicillness.
Dermatologicsignsconsistofthelesionsthemselves:macule,papule,vesicle,etc.Inthis
regard,theclinician’spowersofobservationanddescriptionareacutelychallenged.Patients
oftenpresent for care only after multiple attempts at self-cure using a variety of over-thecounterproducts.The effectsofthose medicinals,aswellasthechronicity ofthecondition,
commonly combine to form a dermatosis that defies recognition and description. In these
situations, foot care providers should look to the periphery of a dermatosis for an early,
representativeprimarylesion.Identificationoftheprimarylesioniskeytoaccuratediagnosis
and successful treatment, and provides the foundation for the algorithm that follows. The
readerisaskedtousethischapterasastarting point,addingandmodifyingthealgorithmas
newconditionsarereportedandolderonesclarified.
MACULARDISEASES
https://t.me/medicina_free
Maculesareidentifiedbytheircolororpigmentarycontrasttoadjacentskin,andshouldbeflat
andnonpalpable.Maculardiseasesaffectingthefeetcanbesubdividedonthebasisofcolor:
brown,red,white,andblue.Ofcourse,oneshouldapplythisschemetoaccountforexpected
variationsinshading(e.g.,lesionslighterthanadjacentskinwouldfallinthe“white”category,
violaceouslesionswithinthe“blue”category,andsoon).
Erythematous
RockyMountainSpottedFever
Rocky Mountain spotted fever (RMSF) typically presents as a triad with fever, rash, and
headache.It presentsdermatologicallyas a maculopapular rashon thehandsandfeetalong
withalatepetechialrashontheforearmandhands.Theexanthemaconsistsofsmallblanching
pinkmacules,locatedinthearches,wrists,andforearms,eventuallyexpandingtotheproximal
extremitiesandtorso.
1–3
This disease has its greatest incidence during the latespringand earlysummer months,
whenticksaremostactiveandactasarthropodvectorsfortheorganismRickettsiarickettsii.
Usually1 weekfollowinga tickbite,RMSFpresentswith anabrupt onsetcharacterizedby
severe headache, fever, chills, nausea, and generalized myalgia. The fourth febrile day is
joinedby theonsetoferythematousmaculesthatstartonthehandsandfeetandthenspread
centrallytothetrunkandface.Twotothreedayslater,themaculesbecomepapularandthen
purpuric, consistent with vasculitis. These same changes occur within viscera, potentially
resultinginshockorrenalfailure.
ThediagnostictoolsusedtoconfirmRMSFareserologictestingandskinbiopsy.Thebest
serologic test is immunofluorescent antibody assays. Also available are enzyme-linked
immunosorbentassaysandlatexagglutinationassays.IfRMSFissuspected,treatmentshould
notbedelayedwhileawaitingconfirmatorytestresults.ThedrugofchoiceusedtotreatRMSF
isdoxycycline.Itisabroadspectrumantibiotic,inthetetracyclinefamily.Whendoxycycline
isadministeredforshortcoursesandunderstrictguidelines,ithasnotbeenfoundtodiscolor
teeth in children <8 years old; however, use in pediatric patients is usually the
contraindication.
Meningococcemia
Althoughpresenting inafashionsimilartoRMSF,the rashofmeningococcemiaoccurs very
rapidly after the onset of constitutional changes. In addition, nuchal rigidity and pain on
passiveflexionofthe neck(secondarytostretching ofpainfulmeninges)provide additional
differentiatingclues.
The widespread ecchymosis and limb ischemia is usually preceded by a petechial or
purpuric rashthat is rapidly spreading.4 Theprocess involves a number of factors that are
initiatedbyabacterialinfectioninvadingthesofttissue,causingswelling,hypoperfusion,and
hypoxia,ultimatelyresultingindisseminatedintravascularcoagulation.
5
Itisrecommendedtohavebloodculturesdrawnpriortothestartoftreatment,andifthe
patientisstable,alumbarpunctureisalsorecommended.Havingsaidthat,therapyshouldnot
https://t.me/medicina_free
bedelayedifthesuspicionishighandthediagnosticproceduresarenotperformed.
5
Thefollowing arethe currenttreatmentoptions5: ceftriaxone 100 mg/kg/d inone totwo
divided doses, cefotaxime 200 mg/kg/d in three divided doses, and penicillin G 500,000
units/kg/dinsixdivideddoses.
Prophylaxis against meningococcus is recommended for those who have been in close
contactwiththepatient.In this case, the drug of choice is rifampin, whichis administered
every12hoursfor2daysor,alternatively,asingledoseofciprofloxacinorceftriaxone.
5
Patientswhopresent with achronicmeningococcemiaposeadiagnosticchallenge.They
usuallypresentwithprolongedperiodsofintermittentfever,arthritisthatismigratory,aswell
asdisseminatedskinlesionsintheformofmaculesornodulesthatareerythematous.6Patients
with chronic meningococcemia may fail to reveal its identity with routine microbiologic
testing.Ithasbeensuggestedinthesecasesthatpolymerasechainreaction(PCR)testingofthe
skin biopsy be performed along with an immunohistochemical approach, sometimes using
silverstainingofthelesions.
6
SecondarySyphilis
The ham-colored maculopapular rash of secondary syphilis usually presents within 3 to 6
weeks afterthe appearanceoftheprimarychancre. The rashis always painless, and never
hemorrhagicorvesiculobullous.Plaques,nodules,andulceratedlesionsarerarebutcanalso
occur. Lesions develop slowly in crops rather than abruptly. Constitutional symptoms are
absent.Thereaderisremindedthatguttatepalmoplantarkeratosesalsoexistinthisstage.
Diagnostic procedures may include HIV, hepatitis B/C, Venereal Disease Research
Laboratory (VDRL), Treponema pallidum particle agglutination test, and anti–T. pallidum
immunoglobulinMenzyme-linkedimmunosorbentassay(ELISA)index.
7,8
Theserologictests
are also combined with immunohistochemical staining of the lesional and nonlesional skin
usingPCRandZiehl–Neelsenstain.
7
The treatment involves giving the patient a prophylactic dose of prednisone to avoid a
Jarisch–Herxheimer reaction. Then treatment would follow with weekly injections of 2.4
millionunitsofbenzathinepenicillin(PCN)forthreeconsecutiveweeks.Itcanalsobedosed
at1millionunitsfor21days,dependingonthespecificguidelinesbeingfollowed.
8
FamilialMediterraneanFever
Oftenconsideredafamilialformofamyloidosiswithchildhoodonset,familialMediterranean
fever(FMF)presentswithhot,tender,erythematouspatchesonthecalves,ankles,orthedorsa
ofthefeet.FMFis a condition thatresults inrecurrent episodes offeverand polyserositis,
whichdoesnotinvolveinfectionorautoantibodies.
9,10
Itisanautoinflammatoryconditionthat
results from a mutation in the MEFV gene, which usually starts during childhood.
9,10
LaboratorytestsmayincludeelevationsinserumamyloidA,erythrocytesedimentationrate,Creactiveprotein,leukocytosis,andfibrinogenduringfebrileepisodes.Definitivediagnosisis
madewithgenetic testingandconfirmedwhenamutationoftheMEFV geneisestablished.
1
Themainstayoftreatmentiscolchicine,butincaseswheretherearepatientsresistanttothe
https://t.me/medicina_free
drug,interferon-αmaybeatreatmentoption.
9,10
MucocutaneousLymphNodeSyndrome(KawasakiDisease)
Kawasakidiseaseisaconditionthattypicallyaffectsinfantsandyoungchildren.Itisknownas
a self-limiting disease causing systemic vasculitis, resulting in coronary artery aneurysms,
myocardial infarctions,and deathifnotpromptly diagnosed and treated.11 Thediagnosis is
based on both clinical and laboratory findings. The following is required to make the
diagnosis, persistent fever >101°F for a minimum of 5 days alongwith at leastfour ofthe
followingcharacteristics:edemaanderythemaoftheextremities;bilateralbulbarconjunctival
injection withoutexudate;edemaof theoropharynxwith lips thatbecome crackedor red, a
“strawberry tongue”; erythematous rash; and cervical lymphadenopathy.11 This disease
demonstrates palmoplantar erythema and pedal edema, followed by a course of marked
desquamationofthepalmsand soles.Thesepatientsoftenhavefeversthatcontinuetospike
despite being treated with antipyretics and antibiotics. The diagnosis may also be made if
feverispersistentfor aminimumof5days,and lessthanfouroftherespectivecriteriaare
met,ifthere are coronaryabnormalitiesfoundon2Decho,oriffourormoreofthecritical
criteria have been established.12 The drug of choice for Kawasaki disease is intravenous
immunoglobulin and acetylsalicylic acid (aspirin) (ASA); steroids and immunosuppressive
therapymayalso bea treatmentoption forthose patients who fail to respondto theinitial
therapy.
11
Graves’Disease
Inadditiontopalmoplantarerythemaandonycholysis,patientsmaypresentwithacombination
ofdiffusegoiter,exophthalmos,pretibialmyxedema, nail clubbing, andperiosteal newbone
formation(thecombinationisoftenreferredtoas“thyroidacropachy”).
GlucagonomaSyndrome
In thisdisorder,an α-cell tumor ofthe pancreas results inincreased serum glucagonlevels
coupledwitherythematousmaculesandpatchesthattransformintoflaccidbullae(Fig.15-1).
Theblisterssubsequentlyrupturetoleave acollaretteofdesquamatingskin.Lesionshavea
predilectionfortheabdomen,groin,thighs,hands,periorificialareas,legs,andfeet.Although
thedermatosisrespondstooraldiiodohydroxyquin,removalofthetumorleadstoresolutionof
theskinchanges.
LeukocytoclasticVasculitis
The lesions may begin as erythematous macules and therefore are included for the sake of
completeness. They rapidly progress to purpuric macules and papules; a more complete
discussionmaybefoundinthesectiononpurpuriclesions.
Nonerythematous
https://t.me/medicina_free
Peutz–JeghersSyndrome
Brownmaculesmaybefoundonthepalms,soles,andmouth(lips)(Fig.15-2).Theseoutward
signsare associatedwithgastrointestinalpolyposis. Historically, treatmentoptionsinvolved
cryotherapy,surgicalremoval, dermabrasion,electric cautery,and theuseofcarbondioxide
andargonlasers.13Alternatively,Lietal.13havestudiedtheuseofaQ-switchedalexandrite
laser in the treatment of both labial and facial lentigines associated with Peutz–Jeghers
syndrome.Their results revealed thatafter threetreatments,55.8% (24/43) oftheir patients
hadexcellentresultsand44%(19/43)hadgoodresults.13Theydidnotdocumentanyserious
complicationsrelatedtotheuseofthelaser.
FIGURE15-1.Erythematousmaculesandpatchestransformintoflaccidbullaeinpatientswiththeα-cell
pancreatictumorassociatedwiththeglucagonomasyndrome(borrowedfromtheMountSinaiCollection
photographs).
https://t.me/medicina_free
Соседние файлы в папке Библиотека им академика М.И. Перельмана