Histopathology of anti-laminin 5 mucous membrane pemphigoid

Background Anti-laminin 5 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the major basement membrane component laminin 5 (laminin 332, epiligrin). Objective and Methods We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 M...

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Veröffentlicht in:Journal of the American Academy of Dermatology 2009-09, Vol.61 (3), p.433-440
Hauptverfasser: Rose, Christian, MD, Schmidt, Enno, MD, PhD, Kerstan, Andreas, MD, Thoma-Uszynski, Sybille, MD, Wesselmann, Ulrich, MD, Käsbohrer, Ulrich, MD, Zillikens, Detlef, MD, Shimanovich, Iakov, MD
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container_end_page 440
container_issue 3
container_start_page 433
container_title Journal of the American Academy of Dermatology
container_volume 61
creator Rose, Christian, MD
Schmidt, Enno, MD, PhD
Kerstan, Andreas, MD
Thoma-Uszynski, Sybille, MD
Wesselmann, Ulrich, MD
Käsbohrer, Ulrich, MD
Zillikens, Detlef, MD
Shimanovich, Iakov, MD
description Background Anti-laminin 5 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the major basement membrane component laminin 5 (laminin 332, epiligrin). Objective and Methods We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 MMP in an attempt to define typical histopathologic features of the disease. Results Fifteen specimens showed subepidermal blister formation, while two biopsy specimens revealed an epithelial ulcer. In 11 biopsies a sparse to moderate inflammatory infiltrate composed of lymphocytes and neutrophils with some eosinophils was observed. Four biopsies showed a dense infiltrate dominated by neutrophils in two cases and by eosinophils in one case. The remaining biopsy revealed a dense lymphoplasmacellular infiltrate without granulocytes. Scarring of the upper dermis was present only in 5 specimens. Immunohistochemical analysis localized type IV collagen to the dermal side of the blister, suggesting that split formation occurred within the lamina lucida of the cutaneous basement membrane. Limitations The number of patients studied was relatively small. Conclusions Histopathology of anti-laminin 5 MMP is characterized by subepidermal blistering and a sparse to moderate superficial lymphohistiocytic infiltrate with neutrophils and/or eosinophils. Both infiltrate density and composition may vary, making anti-laminin 5 MMP indistinguishable from other autoimmune subepidermal blistering diseases by histopathology alone. Scarring is present only in a minority of cases and is not a sensitive clue to the diagnosis of anti-laminin 5 MMP.
doi_str_mv 10.1016/j.jaad.2009.02.012
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Objective and Methods We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 MMP in an attempt to define typical histopathologic features of the disease. Results Fifteen specimens showed subepidermal blister formation, while two biopsy specimens revealed an epithelial ulcer. In 11 biopsies a sparse to moderate inflammatory infiltrate composed of lymphocytes and neutrophils with some eosinophils was observed. Four biopsies showed a dense infiltrate dominated by neutrophils in two cases and by eosinophils in one case. The remaining biopsy revealed a dense lymphoplasmacellular infiltrate without granulocytes. Scarring of the upper dermis was present only in 5 specimens. Immunohistochemical analysis localized type IV collagen to the dermal side of the blister, suggesting that split formation occurred within the lamina lucida of the cutaneous basement membrane. Limitations The number of patients studied was relatively small. Conclusions Histopathology of anti-laminin 5 MMP is characterized by subepidermal blistering and a sparse to moderate superficial lymphohistiocytic infiltrate with neutrophils and/or eosinophils. Both infiltrate density and composition may vary, making anti-laminin 5 MMP indistinguishable from other autoimmune subepidermal blistering diseases by histopathology alone. Scarring is present only in a minority of cases and is not a sensitive clue to the diagnosis of anti-laminin 5 MMP.</description><identifier>ISSN: 0190-9622</identifier><identifier>EISSN: 1097-6787</identifier><identifier>DOI: 10.1016/j.jaad.2009.02.012</identifier><identifier>PMID: 19700013</identifier><identifier>CODEN: JAADDB</identifier><language>eng</language><publisher>New York, NY: Mosby, Inc</publisher><subject>Adult ; Aged ; Autoantibodies - blood ; Basement Membrane - immunology ; Basement Membrane - pathology ; Biological and medical sciences ; Biopsy ; Bullous diseases of the skin ; Cell Adhesion Molecules - immunology ; Child ; Dermatology ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin G - blood ; Kalinin ; Larynx - immunology ; Larynx - pathology ; Male ; Medical sciences ; Mouth - immunology ; Mouth - pathology ; Pemphigoid, Benign Mucous Membrane - immunology ; Pemphigoid, Benign Mucous Membrane - pathology ; Pharynx - immunology ; Pharynx - pathology ; Skin - immunology ; Skin - pathology ; Vulva - immunology ; Vulva - pathology</subject><ispartof>Journal of the American Academy of Dermatology, 2009-09, Vol.61 (3), p.433-440</ispartof><rights>American Academy of Dermatology, Inc.</rights><rights>2009 American Academy of Dermatology, Inc.</rights><rights>2009 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c505t-318d93c874010beb5c0422aab5071e12f70048bc45007acd836b0df32009ccb93</citedby><cites>FETCH-LOGICAL-c505t-318d93c874010beb5c0422aab5071e12f70048bc45007acd836b0df32009ccb93</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0190962209002400$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,65309</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&amp;idt=21876318$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/19700013$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Rose, Christian, MD</creatorcontrib><creatorcontrib>Schmidt, Enno, MD, PhD</creatorcontrib><creatorcontrib>Kerstan, Andreas, MD</creatorcontrib><creatorcontrib>Thoma-Uszynski, Sybille, MD</creatorcontrib><creatorcontrib>Wesselmann, Ulrich, MD</creatorcontrib><creatorcontrib>Käsbohrer, Ulrich, MD</creatorcontrib><creatorcontrib>Zillikens, Detlef, MD</creatorcontrib><creatorcontrib>Shimanovich, Iakov, MD</creatorcontrib><title>Histopathology of anti-laminin 5 mucous membrane pemphigoid</title><title>Journal of the American Academy of Dermatology</title><addtitle>J Am Acad Dermatol</addtitle><description>Background Anti-laminin 5 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the major basement membrane component laminin 5 (laminin 332, epiligrin). Objective and Methods We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 MMP in an attempt to define typical histopathologic features of the disease. Results Fifteen specimens showed subepidermal blister formation, while two biopsy specimens revealed an epithelial ulcer. In 11 biopsies a sparse to moderate inflammatory infiltrate composed of lymphocytes and neutrophils with some eosinophils was observed. Four biopsies showed a dense infiltrate dominated by neutrophils in two cases and by eosinophils in one case. The remaining biopsy revealed a dense lymphoplasmacellular infiltrate without granulocytes. Scarring of the upper dermis was present only in 5 specimens. Immunohistochemical analysis localized type IV collagen to the dermal side of the blister, suggesting that split formation occurred within the lamina lucida of the cutaneous basement membrane. Limitations The number of patients studied was relatively small. Conclusions Histopathology of anti-laminin 5 MMP is characterized by subepidermal blistering and a sparse to moderate superficial lymphohistiocytic infiltrate with neutrophils and/or eosinophils. Both infiltrate density and composition may vary, making anti-laminin 5 MMP indistinguishable from other autoimmune subepidermal blistering diseases by histopathology alone. Scarring is present only in a minority of cases and is not a sensitive clue to the diagnosis of anti-laminin 5 MMP.</description><subject>Adult</subject><subject>Aged</subject><subject>Autoantibodies - blood</subject><subject>Basement Membrane - immunology</subject><subject>Basement Membrane - pathology</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Bullous diseases of the skin</subject><subject>Cell Adhesion Molecules - immunology</subject><subject>Child</subject><subject>Dermatology</subject><subject>Female</subject><subject>Fluorescent Antibody Technique, Direct</subject><subject>Humans</subject><subject>Immunoglobulin G - blood</subject><subject>Kalinin</subject><subject>Larynx - immunology</subject><subject>Larynx - pathology</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Mouth - immunology</subject><subject>Mouth - pathology</subject><subject>Pemphigoid, Benign Mucous Membrane - immunology</subject><subject>Pemphigoid, Benign Mucous Membrane - pathology</subject><subject>Pharynx - immunology</subject><subject>Pharynx - pathology</subject><subject>Skin - immunology</subject><subject>Skin - pathology</subject><subject>Vulva - immunology</subject><subject>Vulva - pathology</subject><issn>0190-9622</issn><issn>1097-6787</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kc1q3TAQhUVpaW6SvkAWwZt2Z3ck_8iipVBCfgqBLpquhSyPE7m25Up24b59x9xLA11kJQTfGR19w9gFh4wDrz72WW9MmwkAlYHIgItXbMdBybSStXzNdsAVpKoS4oSdxtgDgUUu37ITriRdeL5jn-5cXPxslic_-Md94rvETItLBzO6yU1JmYyr9WtMRhybYCZMZhznJ_foXXvO3nRmiPjueJ6xnzfXD1d36f33229XX-9TW0K5pDmvW5XbWhbAocGmtFAIYUxTguTIRUddirqxRQkgjW3rvGqg7fLtX9Y2Kj9jHw5z5-B_rxgXPbpocRioDlXTlSxVXRacQHEAbfAxBuz0HNxowl5z0Jsy3etNmd5GaxCalFHo8jh9bUZsnyNHRwS8PwImWjN0ZMG6-I8TvJYV_ZG4zwcOycUfh0FH63Cy2LqAdtGtdy_3-PJf3A60AnrxF-4x9n4NE1nWXEcK6B_bcrfdggIQBUD-F5lgnRg</recordid><startdate>20090901</startdate><enddate>20090901</enddate><creator>Rose, Christian, MD</creator><creator>Schmidt, Enno, MD, PhD</creator><creator>Kerstan, Andreas, MD</creator><creator>Thoma-Uszynski, Sybille, MD</creator><creator>Wesselmann, Ulrich, MD</creator><creator>Käsbohrer, Ulrich, MD</creator><creator>Zillikens, Detlef, MD</creator><creator>Shimanovich, Iakov, MD</creator><general>Mosby, Inc</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20090901</creationdate><title>Histopathology of anti-laminin 5 mucous membrane pemphigoid</title><author>Rose, Christian, MD ; Schmidt, Enno, MD, PhD ; Kerstan, Andreas, MD ; Thoma-Uszynski, Sybille, MD ; Wesselmann, Ulrich, MD ; Käsbohrer, Ulrich, MD ; Zillikens, Detlef, MD ; Shimanovich, Iakov, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c505t-318d93c874010beb5c0422aab5071e12f70048bc45007acd836b0df32009ccb93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Autoantibodies - blood</topic><topic>Basement Membrane - immunology</topic><topic>Basement Membrane - pathology</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Bullous diseases of the skin</topic><topic>Cell Adhesion Molecules - immunology</topic><topic>Child</topic><topic>Dermatology</topic><topic>Female</topic><topic>Fluorescent Antibody Technique, Direct</topic><topic>Humans</topic><topic>Immunoglobulin G - blood</topic><topic>Kalinin</topic><topic>Larynx - immunology</topic><topic>Larynx - pathology</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Mouth - immunology</topic><topic>Mouth - pathology</topic><topic>Pemphigoid, Benign Mucous Membrane - immunology</topic><topic>Pemphigoid, Benign Mucous Membrane - pathology</topic><topic>Pharynx - immunology</topic><topic>Pharynx - pathology</topic><topic>Skin - immunology</topic><topic>Skin - pathology</topic><topic>Vulva - immunology</topic><topic>Vulva - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Rose, Christian, MD</creatorcontrib><creatorcontrib>Schmidt, Enno, MD, PhD</creatorcontrib><creatorcontrib>Kerstan, Andreas, MD</creatorcontrib><creatorcontrib>Thoma-Uszynski, Sybille, MD</creatorcontrib><creatorcontrib>Wesselmann, Ulrich, MD</creatorcontrib><creatorcontrib>Käsbohrer, Ulrich, MD</creatorcontrib><creatorcontrib>Zillikens, Detlef, MD</creatorcontrib><creatorcontrib>Shimanovich, Iakov, MD</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the American Academy of Dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Rose, Christian, MD</au><au>Schmidt, Enno, MD, PhD</au><au>Kerstan, Andreas, MD</au><au>Thoma-Uszynski, Sybille, MD</au><au>Wesselmann, Ulrich, MD</au><au>Käsbohrer, Ulrich, MD</au><au>Zillikens, Detlef, MD</au><au>Shimanovich, Iakov, MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Histopathology of anti-laminin 5 mucous membrane pemphigoid</atitle><jtitle>Journal of the American Academy of Dermatology</jtitle><addtitle>J Am Acad Dermatol</addtitle><date>2009-09-01</date><risdate>2009</risdate><volume>61</volume><issue>3</issue><spage>433</spage><epage>440</epage><pages>433-440</pages><issn>0190-9622</issn><eissn>1097-6787</eissn><coden>JAADDB</coden><abstract>Background Anti-laminin 5 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the major basement membrane component laminin 5 (laminin 332, epiligrin). Objective and Methods We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 MMP in an attempt to define typical histopathologic features of the disease. Results Fifteen specimens showed subepidermal blister formation, while two biopsy specimens revealed an epithelial ulcer. In 11 biopsies a sparse to moderate inflammatory infiltrate composed of lymphocytes and neutrophils with some eosinophils was observed. Four biopsies showed a dense infiltrate dominated by neutrophils in two cases and by eosinophils in one case. The remaining biopsy revealed a dense lymphoplasmacellular infiltrate without granulocytes. Scarring of the upper dermis was present only in 5 specimens. Immunohistochemical analysis localized type IV collagen to the dermal side of the blister, suggesting that split formation occurred within the lamina lucida of the cutaneous basement membrane. Limitations The number of patients studied was relatively small. Conclusions Histopathology of anti-laminin 5 MMP is characterized by subepidermal blistering and a sparse to moderate superficial lymphohistiocytic infiltrate with neutrophils and/or eosinophils. Both infiltrate density and composition may vary, making anti-laminin 5 MMP indistinguishable from other autoimmune subepidermal blistering diseases by histopathology alone. Scarring is present only in a minority of cases and is not a sensitive clue to the diagnosis of anti-laminin 5 MMP.</abstract><cop>New York, NY</cop><pub>Mosby, Inc</pub><pmid>19700013</pmid><doi>10.1016/j.jaad.2009.02.012</doi><tpages>8</tpages></addata></record>
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subjects Adult
Aged
Autoantibodies - blood
Basement Membrane - immunology
Basement Membrane - pathology
Biological and medical sciences
Biopsy
Bullous diseases of the skin
Cell Adhesion Molecules - immunology
Child
Dermatology
Female
Fluorescent Antibody Technique, Direct
Humans
Immunoglobulin G - blood
Kalinin
Larynx - immunology
Larynx - pathology
Male
Medical sciences
Mouth - immunology
Mouth - pathology
Pemphigoid, Benign Mucous Membrane - immunology
Pemphigoid, Benign Mucous Membrane - pathology
Pharynx - immunology
Pharynx - pathology
Skin - immunology
Skin - pathology
Vulva - immunology
Vulva - pathology
title Histopathology of anti-laminin 5 mucous membrane pemphigoid
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