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Hereditary and Acquired Ichthyosis Vulgaris Workup Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD more... Updated: May 28, 2015 Procedures Skin biopsy specimens can be examined under light microscopy and electron microscopy. Take biopsy samples from regions of maximal hyperkeratosis because areas of mild scaling are less reliable for histopathologic analysis and findings may be indistinguishable from those of normal skin. The thickest scales are usually found on the anterior aspect of the lower leg. Histologic Findings The histological appearance of both hereditary ichthyosis and acquired ichthyosis is practically identical. The stratum corneum shows compact hyperkeratosis, although some areas can be laminated. Follicular plugging may be present and represents associated keratosis pilaris. The granular layer is usually onelayer thick or absent. Ichthyosiform sarcoid, a manifestation of acquired ichthyosis in sarcoid patients, has the additional presence of multiple noncaseating granulomas in the dermis. Note the image below. Hematoxylin and eosin staining of acquired ichthyosis vulgaris in an adult. Show s epidermal atrophy w ith thinning of the granular layer. No dermal infiltrate is evident. Ultrastructural studies show reduced or absent keratohyalin granules housed in the granular layer. They appear spongy or crumbly, most likely due to defective keratohyalin synthesis. The hyperkeratotic portions of the stratum corneum have a normal keratin pattern. Contributor Information and Disclosures Author Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi Disclosure: Nothing to disclose. Coauthor(s) Jason F Okulicz, MD, FACP, FIDSA Director, HIV Medical Evaluation Unit, Infectious Disease Service, San Antonio Military Medical Center; Associate Professor of Medicine, F Edward Hebert School of Medicine, Uniformed Services University of the Health Sciences; Clinical Associate Professor of Medicine, University of Texas Health Science Center at San Antonio; Adjunct Clinical Instructor, Feik School of Pharmacy, University of the Incarnate Word Jason F Okulicz, MD, FACP, FIDSA is a member of the following medical societies: Alpha Omega Alpha, American College of PhysiciansAmerican Society of Internal Medicine, Infectious Diseases Society of America Disclosure: Nothing to disclose. Specialty Editor Board David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa Disclosure: Nothing to disclose. Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

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  • 6/17/2015 HereditaryandAcquiredIchthyosisVulgarisWorkup:Procedures,HistologicFindings

    http://emedicine.medscape.com/article/1112753workup#showall 1/3

    HereditaryandAcquiredIchthyosisVulgarisWorkupAuthor:RobertASchwartz,MD,MPHChiefEditor:DirkMElston,MDmore...

    Updated:May28,2015

    ProceduresSkinbiopsyspecimenscanbeexaminedunderlightmicroscopyandelectronmicroscopy.Takebiopsysamplesfromregionsofmaximalhyperkeratosisbecauseareasofmildscalingarelessreliableforhistopathologicanalysisandfindingsmaybeindistinguishablefromthoseofnormalskin.Thethickestscalesareusuallyfoundontheanterioraspectofthelowerleg.

    HistologicFindingsThehistologicalappearanceofbothhereditaryichthyosisandacquiredichthyosisispracticallyidentical.Thestratumcorneumshowscompacthyperkeratosis,althoughsomeareascanbelaminated.Follicularpluggingmaybepresentandrepresentsassociatedkeratosispilaris.Thegranularlayerisusuallyonelayerthickorabsent.Ichthyosiformsarcoid,amanifestationofacquiredichthyosisinsarcoidpatients,hastheadditionalpresenceofmultiplenoncaseatinggranulomasinthedermis.Notetheimagebelow.

    Hematoxylinandeosinstainingofacquiredichthyosisvulgarisinanadult.Showsepidermalatrophyw iththinningofthegranularlayer.Nodermalinfiltrateisevident.

    Ultrastructuralstudiesshowreducedorabsentkeratohyalingranuleshousedinthegranularlayer.Theyappearspongyorcrumbly,mostlikelyduetodefectivekeratohyalinsynthesis.Thehyperkeratoticportionsofthestratumcorneumhaveanormalkeratinpattern.

    ContributorInformationandDisclosuresAuthorRobertASchwartz,MD,MPHProfessorandHeadofDermatology,ProfessorofPathology,Pediatrics,Medicine,andPreventiveMedicineandCommunityHealth,RutgersNewJerseyMedicalSchoolVisitingProfessor,RutgersUniversitySchoolofPublicAffairsandAdministration

    RobertASchwartz,MD,MPHisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,NewYorkAcademyofMedicine,AmericanAcademyofDermatology,AmericanCollegeofPhysicians,SigmaXi

    Disclosure:Nothingtodisclose.

    Coauthor(s)JasonFOkulicz,MD,FACP,FIDSADirector,HIVMedicalEvaluationUnit,InfectiousDiseaseService,SanAntonioMilitaryMedicalCenterAssociateProfessorofMedicine,FEdwardHebertSchoolofMedicine,UniformedServicesUniversityoftheHealthSciencesClinicalAssociateProfessorofMedicine,UniversityofTexasHealthScienceCenteratSanAntonioAdjunctClinicalInstructor,FeikSchoolofPharmacy,UniversityoftheIncarnateWord

    JasonFOkulicz,MD,FACP,FIDSAisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanCollegeofPhysiciansAmericanSocietyofInternalMedicine,InfectiousDiseasesSocietyofAmerica

    Disclosure:Nothingtodisclose.

    SpecialtyEditorBoardDavidFButler,MDSectionChiefofDermatology,CentralTexasVeteransHealthcareSystemProfessorofDermatology,TexasA&MUniversityCollegeofMedicineFoundingChair,DepartmentofDermatology,ScottandWhiteClinic

    DavidFButler,MDisamemberofthefollowingmedicalsocieties:AmericanMedicalAssociation,AlphaOmegaAlpha,AssociationofMilitaryDermatologists,AmericanAcademyofDermatology,AmericanSocietyforDermatologicSurgery,AmericanSocietyforMOHSSurgery,PhiBetaKappa

    Disclosure:Nothingtodisclose.

    LesterFLibow,MDDermatopathologist,SouthTexasDermatopathologyLaboratory

    LesterFLibow,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatology,AmericanSocietyofDermatopathology,TexasMedicalAssociation

  • 6/17/2015 HereditaryandAcquiredIchthyosisVulgarisWorkup:Procedures,HistologicFindings

    http://emedicine.medscape.com/article/1112753workup#showall 2/3

    Disclosure:Nothingtodisclose.

    ChiefEditorDirkMElston,MDDirector,AckermanAcademyofDermatopathology,NewYork

    DirkMElston,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatology

    Disclosure:Nothingtodisclose.

    AdditionalContributorsAlbertCYan,MDSectionChief,AssociateProfessor,DepartmentofPediatrics,SectionofDermatology,Children'sHospitalofPhiladelphiaandUniversityofPennsylvaniaSchoolofMedicine

    AlbertCYan,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofDermatology,SocietyforInvestigativeDermatology,SocietyforPediatricDermatology,AmericanAcademyofPediatrics

    Disclosure:Nothingtodisclose.

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