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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutaneous autoimmune disorders exist on a biological spectrum&#46; A conceptually challenging condition is <span class="elsevierStyleItalic">Lichen Planus Pemphigoides</span> &#40;LPP&#41;&#44; cases of which appear to share features of bullous pemphigoid and lichen planus&#46; Herein we present three recent cases and emphasize classification as LPP can be made using clinical features in conjunction with histological and immunofluorescence findings&#46; More specifically&#44; classification as LPP can be made in the context of 1&#41; Lichenoid lesions clinically and histologically&#44; 2&#41; Linear staining along the basement membrane zone &#40;BMZ&#41; of IgG and&#47;or C3 on immunofluorescence studies&#44; and 3&#41; Lack of evidence to support another specific diagnosis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Clinical descriptions of LPP commonly include lichen planus-like lesions with the additional finding of tense blisters and bullae&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The histology is said to be lichen planus-like&#46; Positive immunofluorescence showing deposition along the dermal-epidermal junction is considered a sine qua non-feature&#46; A number of studies have found the autoantigen to be directed against the NC16A subdomain of collagen XVII &#40;BP180&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However&#44; significant heterogeneity in specific target antigen&#40;s&#41; has been documented&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Classification criteria are used to help group conditions for the study&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> They are not meant to serve as diagnostic criteria but are often used at a practical level by emphasizing important disease features&#46; Notably&#44; because diagnostic criteria are limited by inherent sensitivity and specificity characteristics&#44; classification criteria are published by the American College of Rheumatology&#44; whereas diagnostic criteria are not&#46; Given the historic controversy associated with LPP&#44; this is a disease for which classification criteria-like guidelines would be clinically useful&#46;<span class="elsevierStyleEnunciation" id="enun0005"><span class="elsevierStyleLabel">Case 1</span><p id="par0020" class="elsevierStylePara elsevierViewall">was a 55-year-old male with untreated colonic adenocarcinoma who presented with a pruritic rash consisting of violaceous scaly papules and plaques involving the extremities and trunk for several months with more recent blistering &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#8210;B&#41;&#46; Biopsy of a representative lichenoid lesion revealed a brisk lichenoid interface dermatitis histologically consistent with lichen planus &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41;&#46; Perilesional biopsy for Direct Immunofluorescence &#40;DIF&#41; revealed linear C3 deposition without accompanying IgG &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>D&#41;&#44; cytoid bodies and shaggy&#44; fibrillar fibrinogen deposition at the BMZ&#46; The patient improved on prednisone&#44; without recurrence after the taper&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span class="elsevierStyleEnunciation" id="enun0010"><span class="elsevierStyleLabel">Case 2</span><p id="par0025" class="elsevierStylePara elsevierViewall">was a 29-year-old female who presented with a 6-month history of lichenoid papules and plaques&#44; presumptively diagnosed as lichen planus&#46; At presentation&#44; she was also noted to have scattered vesicles and bullae overlying unaffected and lichenoid skin&#44; distributed on the extremities and trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#8210;B&#41;&#46; The autoantibody workup was negative&#44; and no systemic symptoms were present&#46; A biopsy revealed a robust interface process with features of lichen planus&#46; However&#44; in contrast to typical LP the epidermis was completely detached from the underlying dermis&#44; forming a blistering space histologically &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C&#41;&#46; DIF showed faint linear IgG&#44; intense linear deposition of C3 &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>D&#41;&#44; and fibrinogen along the BMZ with junctional cytoid bodies&#46; She was started on prednisone and methotrexate with good disease control at follow-up&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span class="elsevierStyleEnunciation" id="enun0015"><span class="elsevierStyleLabel">Case 3</span><p id="par0030" class="elsevierStylePara elsevierViewall">was a 68-year-old female who presented with a 2-month history of a scaly pruritic rash&#46; Outside biopsy at the onset of her rash revealed a band-like infiltrate of lymphocytes in the papillary dermis with vacuolar change and necrotic keratinocytes&#44; histologically consistent with lichen planus&#46; One week prior to their presentation in our clinic she additionally developed bullae over the upper extremities and trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A&#8210;B&#41;&#46; DIF revealed linear C3 deposition at the BMZ without IgG&#46; Indirect immunofluorescence was positive for linear IgG staining localizing to the epidermal side of salt-split skin at a titer of 1&#58;1280&#46; She was treated with triamcinolone wraps&#44; prednisone&#44; and intravenous immunoglobulin with improvement&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span></p><p id="par0035" class="elsevierStylePara elsevierViewall">In all three cases&#44; we were able to exclude traditional lichen planus because of the immunofluorescence findings&#46; Traditional bullous pemphigoid was excluded because of lichenoid histological and clinical findings&#46; None of the cases had other evidence of lupus or bullous lupus erythematosus&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Autoimmune disorders exist on a biological spectrum and often no solitary test has absolute specificity&#46; In the past&#44; authors have noted cases that share features of both lichen planus and bullous pemphigoid and have termed these cases <span class="elsevierStyleItalic">lichen planus pemphigoid</span>&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In accordance with our concept of the disease and review of the literature&#44; we emphasize the following classification guidelines&#58; 1&#41; Lichenoid lesions clinically and histologically&#44; precluding classification as routine lichen planus or bullous pemphigoid&#59; 2&#41; Linear staining along the BMZ of IgG and&#47;or C3 on immunofluorescence studies&#59; 3&#41; Lack of diagnostic findings for a separate specific diagnosis&#44; such as lupus erythematosus&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Notably&#44; the above omits the need for more exotic ancillary testing&#44; such as enzyme-linked immunosorbent assay or antigen subtyping&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">We choose the term &#8220;classification guidelines&#8221; in an attempt to parallel other conditions in rheumatology&#46; Challenges in classification are reflected in the American College of Rheumatology&#8217;s publication of classification criteria&#44; based on the recognition that rheumatic diseases &#8220;tend to be heterogenous in their presentation&#44; course&#44; and outcome and not have a single clinical&#44; laboratory&#44; pathologic&#44; or radiologic feature that could serve as a &#8220;gold standard&#8230;&#46;&#8221;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">LPP is not a commonly made diagnosis&#46; Zaraa et al&#46; published the largest case review&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> They conceptualized the cases as a combination between lichen planus and bullous pemphigoid and emphasize the importance of clinical and histological correlation&#46; Hubner et al&#46; also underscored the importance of careful clinicopathological correlation&#44; especially to exclude other entities&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">When presented with similar cases&#44; some clinicians may experience confusion in nosological classification&#46; However&#44; we feel the emphasis of these criteria may help clinicians feel confident in the appropriateness of a diagnosis of lichen planus pemphigoid&#44; when these minimum criteria are satisfied&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#8217; contributions</span><p id="par0075" class="elsevierStylePara elsevierViewall">Reed Maggard&#58; Approval of the final manuscript version&#59; critical literature review&#59; manuscript critical review&#59; preparation and writing of manuscript&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Donna A&#46; Culton&#58; Approval of the final manuscript version&#59; critical literature review&#59; data collection&#44; analysis and interpretation&#59; intellection participation and&#47;or therapeutic management of studied cases&#59; manuscript critical review&#59; study concept and planning&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Amy Blake&#58; Approval of final version of the manuscript&#59; intellection participation and&#47;or therapeutic management of studied cases&#59; data collection&#44; analysis and interpretation&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Paul Googe&#58; Approval of the final version of the manuscript&#59; data collection analysis and interpretation&#59; intellectual participation and&#47;or therapeutic management of studied cases&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Jayson Miedema&#58; Approval of the final version of the manuscript&#59; critical literature review&#59; intellectual participation and&#47;or therapeutic management of studied cases&#59; preparation and writing of manuscript&#59; manuscript critical review&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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Letter - Research
What is Lichen planus pemphigoides? A highlight of three cases with discussion of differential diagnosis and suggestion of simple classification guidelines
Reed Maggarda, Donna A. Cultonb, Amy Blakeb, Paul Googeb,c, Jayson Miedemab,c,
Corresponding author
jayson_miedema@med.unc.edu

Corresponding author.
a University of Washington School of Medicine, Seattle, WA, USA
b Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
c Department of Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Cutaneous autoimmune disorders exist on a biological spectrum&#46; A conceptually challenging condition is <span class="elsevierStyleItalic">Lichen Planus Pemphigoides</span> &#40;LPP&#41;&#44; cases of which appear to share features of bullous pemphigoid and lichen planus&#46; Herein we present three recent cases and emphasize classification as LPP can be made using clinical features in conjunction with histological and immunofluorescence findings&#46; More specifically&#44; classification as LPP can be made in the context of 1&#41; Lichenoid lesions clinically and histologically&#44; 2&#41; Linear staining along the basement membrane zone &#40;BMZ&#41; of IgG and&#47;or C3 on immunofluorescence studies&#44; and 3&#41; Lack of evidence to support another specific diagnosis&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Clinical descriptions of LPP commonly include lichen planus-like lesions with the additional finding of tense blisters and bullae&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The histology is said to be lichen planus-like&#46; Positive immunofluorescence showing deposition along the dermal-epidermal junction is considered a sine qua non-feature&#46; A number of studies have found the autoantigen to be directed against the NC16A subdomain of collagen XVII &#40;BP180&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> However&#44; significant heterogeneity in specific target antigen&#40;s&#41; has been documented&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;6</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">Classification criteria are used to help group conditions for the study&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> They are not meant to serve as diagnostic criteria but are often used at a practical level by emphasizing important disease features&#46; Notably&#44; because diagnostic criteria are limited by inherent sensitivity and specificity characteristics&#44; classification criteria are published by the American College of Rheumatology&#44; whereas diagnostic criteria are not&#46; Given the historic controversy associated with LPP&#44; this is a disease for which classification criteria-like guidelines would be clinically useful&#46;<span class="elsevierStyleEnunciation" id="enun0005"><span class="elsevierStyleLabel">Case 1</span><p id="par0020" class="elsevierStylePara elsevierViewall">was a 55-year-old male with untreated colonic adenocarcinoma who presented with a pruritic rash consisting of violaceous scaly papules and plaques involving the extremities and trunk for several months with more recent blistering &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#8210;B&#41;&#46; Biopsy of a representative lichenoid lesion revealed a brisk lichenoid interface dermatitis histologically consistent with lichen planus &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>C&#41;&#46; Perilesional biopsy for Direct Immunofluorescence &#40;DIF&#41; revealed linear C3 deposition without accompanying IgG &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>D&#41;&#44; cytoid bodies and shaggy&#44; fibrillar fibrinogen deposition at the BMZ&#46; The patient improved on prednisone&#44; without recurrence after the taper&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span class="elsevierStyleEnunciation" id="enun0010"><span class="elsevierStyleLabel">Case 2</span><p id="par0025" class="elsevierStylePara elsevierViewall">was a 29-year-old female who presented with a 6-month history of lichenoid papules and plaques&#44; presumptively diagnosed as lichen planus&#46; At presentation&#44; she was also noted to have scattered vesicles and bullae overlying unaffected and lichenoid skin&#44; distributed on the extremities and trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A&#8210;B&#41;&#46; The autoantibody workup was negative&#44; and no systemic symptoms were present&#46; A biopsy revealed a robust interface process with features of lichen planus&#46; However&#44; in contrast to typical LP the epidermis was completely detached from the underlying dermis&#44; forming a blistering space histologically &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>C&#41;&#46; DIF showed faint linear IgG&#44; intense linear deposition of C3 &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>D&#41;&#44; and fibrinogen along the BMZ with junctional cytoid bodies&#46; She was started on prednisone and methotrexate with good disease control at follow-up&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span class="elsevierStyleEnunciation" id="enun0015"><span class="elsevierStyleLabel">Case 3</span><p id="par0030" class="elsevierStylePara elsevierViewall">was a 68-year-old female who presented with a 2-month history of a scaly pruritic rash&#46; Outside biopsy at the onset of her rash revealed a band-like infiltrate of lymphocytes in the papillary dermis with vacuolar change and necrotic keratinocytes&#44; histologically consistent with lichen planus&#46; One week prior to their presentation in our clinic she additionally developed bullae over the upper extremities and trunk &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A&#8210;B&#41;&#46; DIF revealed linear C3 deposition at the BMZ without IgG&#46; Indirect immunofluorescence was positive for linear IgG staining localizing to the epidermal side of salt-split skin at a titer of 1&#58;1280&#46; She was treated with triamcinolone wraps&#44; prednisone&#44; and intravenous immunoglobulin with improvement&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span></p><p id="par0035" class="elsevierStylePara elsevierViewall">In all three cases&#44; we were able to exclude traditional lichen planus because of the immunofluorescence findings&#46; Traditional bullous pemphigoid was excluded because of lichenoid histological and clinical findings&#46; None of the cases had other evidence of lupus or bullous lupus erythematosus&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Autoimmune disorders exist on a biological spectrum and often no solitary test has absolute specificity&#46; In the past&#44; authors have noted cases that share features of both lichen planus and bullous pemphigoid and have termed these cases <span class="elsevierStyleItalic">lichen planus pemphigoid</span>&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In accordance with our concept of the disease and review of the literature&#44; we emphasize the following classification guidelines&#58; 1&#41; Lichenoid lesions clinically and histologically&#44; precluding classification as routine lichen planus or bullous pemphigoid&#59; 2&#41; Linear staining along the BMZ of IgG and&#47;or C3 on immunofluorescence studies&#59; 3&#41; Lack of diagnostic findings for a separate specific diagnosis&#44; such as lupus erythematosus&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Notably&#44; the above omits the need for more exotic ancillary testing&#44; such as enzyme-linked immunosorbent assay or antigen subtyping&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">We choose the term &#8220;classification guidelines&#8221; in an attempt to parallel other conditions in rheumatology&#46; Challenges in classification are reflected in the American College of Rheumatology&#8217;s publication of classification criteria&#44; based on the recognition that rheumatic diseases &#8220;tend to be heterogenous in their presentation&#44; course&#44; and outcome and not have a single clinical&#44; laboratory&#44; pathologic&#44; or radiologic feature that could serve as a &#8220;gold standard&#8230;&#46;&#8221;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">LPP is not a commonly made diagnosis&#46; Zaraa et al&#46; published the largest case review&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> They conceptualized the cases as a combination between lichen planus and bullous pemphigoid and emphasize the importance of clinical and histological correlation&#46; Hubner et al&#46; also underscored the importance of careful clinicopathological correlation&#44; especially to exclude other entities&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">When presented with similar cases&#44; some clinicians may experience confusion in nosological classification&#46; However&#44; we feel the emphasis of these criteria may help clinicians feel confident in the appropriateness of a diagnosis of lichen planus pemphigoid&#44; when these minimum criteria are satisfied&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0070" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#8217; contributions</span><p id="par0075" class="elsevierStylePara elsevierViewall">Reed Maggard&#58; Approval of the final manuscript version&#59; critical literature review&#59; manuscript critical review&#59; preparation and writing of manuscript&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">Donna A&#46; Culton&#58; Approval of the final manuscript version&#59; critical literature review&#59; data collection&#44; analysis and interpretation&#59; intellection participation and&#47;or therapeutic management of studied cases&#59; manuscript critical review&#59; study concept and planning&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Amy Blake&#58; Approval of final version of the manuscript&#59; intellection participation and&#47;or therapeutic management of studied cases&#59; data collection&#44; analysis and interpretation&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Paul Googe&#58; Approval of the final version of the manuscript&#59; data collection analysis and interpretation&#59; intellectual participation and&#47;or therapeutic management of studied cases&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Jayson Miedema&#58; Approval of the final version of the manuscript&#59; critical literature review&#59; intellectual participation and&#47;or therapeutic management of studied cases&#59; preparation and writing of manuscript&#59; manuscript critical review&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0100" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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