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1、DermalandSubcutaneousTumorsMastocytosis urticariapigmentosaLocalandsystemicaccumulationsofmastcellsPersistentpigmenteditchyskinlesionsUrticateonmechanicalorchemicalirritationc-KITmutationBirthtomiddleage,½<6moMacules,papules,nodules,plaques,vesiclesLesionspersistandgr
2、aduallybecomechamois-orslate-coloredDarier’ssign,pruritisSeveresymptomsmayresultfrommassiveliberationofhistaminefrommastcellsafteringestionofknownmastcelldegranulatorsSpontaneousresolutionislikelyinthoseptswhosediseasebeganinchildhoodUrticariapigmentosaSolitarymastocy
3、tomaMaybepresentatbirth,maydevelopduringthefirstweeksoflifeBrownmaculethaturticatesuponstrokingSmoothorpeaud’orangeDorsumofthehandnearthewristEdema,urtication,vesiculationmaybeobservedGeneralizederuption,childhoodtypePseudoxanthomatousmastocytosisDiffusecutaneousmasto
4、cytosisGeneralizederuption,adulttypeErythrodermicmastocytosisTelangiectasiamaculariseruptaperstansSystemicmastocytosisFamilialurticariapigmentosaGiemsa,azureA,orpolychrometoluidineblueLocalanestheticadjacenttothelesion,withoutepiDxisbxconfirmedHistaminemetabolitesmeth
5、ylhistamineandmethylimidazoleaceticacidPrognosisandtreatmentInallformswithoutsystemicinvolvementtheprognosisisgoodSolitarylesionsusuallyinvolutewithin3yearsH1andH2blockersPUVAIntralesionalandtopicalsteroidsAvoidphysicalstimuliAbnormalitiesofneuraltissueSolitaryneurofi
6、bromaSoft,flaccid,pinkishwhite,2-20mmInvaginatesonpressure,“buttonholing”SolitaryormultipleDistinctivehistopathologicfindings,fibrils,cellularproliferation,anddegenerativechangesSxexcisionGranularcelltumorWell-circumscribed,solitaryfirmnodule,withabrownishredorfleshti
7、ntUsuallysolitary,10-15%multiple1/3ofcaseshaveoccurredonthetongueMayoccuranywhereonthebodyGrowsslowlyCellsstainpositivelywithvimentin,neuron-specificenolase,S-100,andmyelinproteinMalignantgranularcelltumorisrareNeuromacutisThreetrueneuromasexistintheskinandmucousmembr
8、anes:traumaticneuroma,multiplemucosalneuromas,andsolitarypalisadedencapsulatedneuromasTraumaticneuromasoccurcommonlyonthefin
