medicina Communication Diagnosis of Laryngeal Pemphigus Vulgaris Can Be Facilitated Using Advanced Endoscopic Methods Lucia Staníková 1, Martin Formánek 1,2 , Pavel Hurník 3, Peter Kántor 1,2 , Pavel Komínek 1,2 and Karol Zeleník 1,2,* 1 Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Ostrava, 17. Listopadu 1790, 70800 Ostrava, Czech Republic; [email protected] (L.S.); [email protected] (M.F.); [email protected] (P.K.); [email protected] (P.K.) 2 Department of Craniofacial Surgery, Faculty of Medicine, University of Ostrava, Syllabova 19, 70300 Ostrava, Czech Republic 3 Department of Pathology, University Hospital Ostrava, 17. listopadu 1790, 70800 Ostrava, Czech Republic; [email protected] * Correspondence: [email protected] Abstract: Background: Isolated laryngeal pemphigus vulgaris (LPV) is rare; however, early diagnosis is crucial in determining its course and prognosis. This paper aims to describe mucosal vascular changes typical for LPV using advanced endoscopic methods, which include Narrow Band Imaging (NBI), IMAGE1-S video-endoscopy and enhanced contact endoscopy (ECE). Materials and Methods: Retrospective analysis of all laryngeal mucosal lesion examined using advanced endoscopic methods during 2018–2020 at tertiary hospital was performed. Results: Videolaryngoscopy examination records of 278 patients with laryngeal mucosal lesions were analyzed; three of them were diagnosed Citation: Staníková, L.; Formánek, with LPV. Epithelial vascularization of LPV included specific pattern. Intraepithelial papillary M.; Hurník, P.; Kántor, P.; Komínek, P.; capillary loops were symmetrically stratified and were organized into “contour-like lines”. This Zeleník, K. Diagnosis of Laryngeal specific vascularization associated with LPV were different from other laryngeal mucosal pathologies. Pemphigus Vulgaris Can Be Conclusions: Using advanced endoscopic methods supports early diagnosis of LPV and accelerate the Facilitated Using Advanced diagnosis and treatment. Endoscopic Methods. Medicina 2021, 57, 686. https://doi.org/10.3390/ medicina57070686 Keywords: pemphigus vulgaris; larynx; Narrow Band Imaging (NBI); IMAGE1-S; enhanced contact endoscopy (ECE) Academic Editors: Sylvia Hsu and Kiran Motaparthi Received: 17 June 2021 1. Introduction Accepted: 4 July 2021 Pemphigus comprises a group of autoimmune blistering diseases with skin and Published: 6 July 2021 mucosal manifestations. Pemphigus vulgaris (PV) is a mucocutaneous disease that presents as enanthemas and erosions, typically in the oral cavity. Other mucosal surfaces are less Publisher’s Note: MDPI stays neutral frequently involved [1]. Exfoliating blisters are painful, can bleed, and in severe cases, with regard to jurisdictional claims in they can lead to rapid weight loss. Pemphigus may have an idiopathic origin, or in published maps and institutional affil- some cases may be a drug-induced reaction [2]. Rarely, particularly in patients with iations. lymphoproliferative disorders (mostly non-Hodgkin lymphoma and chronic lymphocytic leukemia), PV is considered a paraneoplastic manifestation [1]. PV with primary laryngeal involvement and without other mucosal or skin lesions is extremely rare; to date, only a very limited number of case series and case reports have been Copyright: © 2021 by the authors. published on this manifestation [3–8]. Laryngeal mucosal lesions, like leukoplakia and Licensee MDPI, Basel, Switzerland. erythroplakia, typically arouse suspicion of a precancerous or cancerous lesion. Laryngeal This article is an open access article pemphigus vulgaris (LPV) is not typically considered first in a differential diagnosis, distributed under the terms and although early diagnosis and treatment affect its course and prognosis. conditions of the Creative Commons It has been acknowledged that advanced endoscopy methods, like narrow band Attribution (CC BY) license (https:// imaging (NBI), IMAGE1-S™ video-endoscopy and enhanced contact endoscopy (ECE), creativecommons.org/licenses/by/ can facilitate the pre-histological diagnosis of mucosal lesions [9–18]. However, to date, no 4.0/). Medicina 2021, 57, 686. https://doi.org/10.3390/medicina57070686 https://www.mdpi.com/journal/medicina MedicinaMedicina 2021,57 57, x FOR PEER REVIEW 2 of 6 2021, , 686 2 of 6 studystudy hashas describeddescribed a a vascular vascular pattern pattern typical typical of of LPV. LPV. Therefore, Therefore, this this short short communication communica- aimedtion aimed to describe to describe the vascular the vascular pattern pattern typical typical of LPV ofand LPV discuss and discuss differences differences fromother from laryngealother laryngeal pathologies. pathologies. 2.2. MaterialsMaterials andand MethodsMethods RetrospectiveRetrospective analysisanalysis ofof allall flexibleflexible videolaryngoscopyvideolaryngoscopy examination records per- formedformed withwith aimaim toto evaluateevaluate laryngeallaryngeal mucosalmucosal lesionslesions duringduring 20182018 toto 20202020 periodperiod waswas performed.performed. AfterAfter flexibleflexible videolaryngoscopy,videolaryngoscopy, allall patientspatients underwentunderwent microlaryngoscopymicrolaryngoscopy withwith excisionexcision ofof mucosalmucosal lesionlesion andand itsits histopathologyhistopathology examinations.examinations. Patients with diag- nosednosed LPVLPV werewere selectedselected andand theirtheir videolaryngoscopyvideolaryngoscopy examinationexamination recordsrecords (including(including advancedadvanced endoscopyendoscopy methods) methods) were were compared compared with with other other findings findings to to determine determine if vascularif vascu- patternlar pattern of LPV of LPV differs differs or not. or not. 3.3. ResultsResults VideolaryngoscopyVideolaryngoscopy examination examination records records of of 278 278 patients patients with with laryngeal laryngeal mucosal mucosal lesions le- weresions analyzed;were analyzed; 3 of them 3 of werethem diagnosed were diagnosed with LPV with (Figure LPV (1Figure). 1). FigureFigure 1.1. HistopathologyHistopathology and direct immunofluorescenceimmunofluorescence of laryngeal pemphigus vulgaris speci- spec- imens.mens. Upper Upper left left—suprabasilar—suprabasilar acantholytic acantholytic blister blister with with intraepithelial intraepithelial suprabasilar suprabasilar clefting; clefting; HE stain, 100× magnification. Upper right—suprabasilar acantholysis and plasma cell infiltrate among HE stain, 100× magnification. Upper right—suprabasilar acantholysis and plasma cell infiltrate villous-like projections of lamina propria, covered by a few layers of epithelium; HE stain, 200× among villous-like projections of lamina propria, covered by a few layers of epithelium; HE stain, magnification. Lower left—intercellular linear deposition of IgG in direct immunofluorescence, DIF × 200IgG, 200magnification.× magnificationLower. Lower left—intercellular right—intercellular linear granular deposition deposition of IgG of in C3 direct in direct immunofluores- immunoflu- cence,orescence, DIF IgG,DIF C3, 200 ×200magnification.× magnification Lower. right—intercellular granular deposition of C3 in direct immunofluorescence, DIF C3, 200× magnification. Epithelial vascularization of mucosal lesions in patients with LPV included specific Epithelial vascularization of mucosal lesions in patients with LPV included specific pattern. LPV manifested as flat lesions that rise slightly above the mucosal surface. The pattern. LPV manifested as flat lesions that rise slightly above the mucosal surface. The surface of LPV lesions was partly coated with different thicknesses of leukoplakia. In areas surface of LPV lesions was partly coated with different thicknesses of leukoplakia. In areas of little or no leukoplakia, intraepithelial papillary capillary loops (IPCLs), symmetrically of little or no leukoplakia, intraepithelial papillary capillary loops (IPCLs), symmetrically stratified and organized into contour-like lines, were observed with advanced endoscopic stratified and organized into contour-like lines, were observed with advanced endoscopic imaging (NBI, IMAGE1-S). IPCLs were very thin and short, and they ran towards the sur- imaging (NBI, IMAGE1-S). IPCLs were very thin and short, and they ran towards the face. They appeared as small brown spots, symmetrically distributed, aligned in regular surface. They appeared as small brown spots, symmetrically distributed, aligned in regular rows and without clear boundaries. The overall picture resembles a “contour line map” rows and without clear boundaries. The overall picture resembles a “contour line map” (Figures 2–4). This appearance is very specific and was not observed in other laryngeal Medicina 2021, 57, 686 3 of 6 Medicina 2021, 57, x FOR PEER REVIEW 3 of 6 Medicina 2021, 57, x FOR PEER REVIEW 3 of 6 (Figures2–4). This appearance is very specific and was not observed in other laryngeal pathologiespathologies (laryngeal(laryngeal cancer,cancer, dysplasticdysplastic changes,changes, papillomatosis,papillomatosis, polypspolyps etc.)etc.) inin ourour groupgrouppathologies ofof patients.patients. (laryngeal EnhancedEnhanced cancer, contactcontact dysplastic endoscopyendoscopy changes, (ECE)(ECE) papillomatosis, revealedrevealed thesethese changeschangespolyps etc.) eveneve nin moremore our preciselypreciselygroup of (Figure(patients.Figure5 ).5 ).Enhanced contact endoscopy (ECE) revealed these changes even more precisely (Figure 5). FigureFigure 2.2. PatientPatient 1,1, epiglottis.epiglottis.