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Mucocutaneous Lesions in an Adult Man Who Recently Moved From Mexico

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 66-year-old man presented to the emergency department for painful and pruritic ulcerative cutaneous lesions involving his back, nose, lips (Figure, A), and mucosal lesions involving his oral cavity and oropharynx. The lesions initially only involved his lips and nasal bridge, and appeared 5 months prior while he was living on a ranch in Mexico. The lesions did not respond to treatment with an unknown oral antibiotic. His medical history included hypertension treated with captopril and a remote history of psoriasis. Physical examination demonstrated several dry, crusted skin lesions with blistering and ulceration, and shallow erythematous ulcerations of his oral mucosa. Internal medicine, otolaryngology, and dermatology departments were consulted. Results of flexible fiberoptic laryngoscopy demonstrated erythematous and ulcerative lesions with surrounding leukoplakia involving the base of tongue, glossoepiglottic fold, lingual and laryngeal surfaces of the epiglottis, and aryepiglottic folds (Figure, C and D). A QuantiFERON-TB Gold test was obtained, and results were found to be positive. Serum antigen testing for blastomyces, histoplasmosis, syphilis, HIV, hepatitis B virus, hepatitis C virus, human herpesvirus 6, cytomegalovirus, Epstein-Barr virus, varicella zoster virus, human polyomavirus 2, and West Nile virus were performed, and all results were found to be negative. A biopsy of the skin lesion was obtained from the edge of the lesion found on the patient’s back (Figure, B). Enzyme-linked immunosorbent assay serological testing was subsequently performed, and results demonstrated elevated levels of antidesmoglein 1 immunoglobulin G (IgG) and antidesmoglein 3 IgG.

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C. Pemphigus vulgaris

Pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease that is clinically characterized by erosions involving mucosal membranes with or without skin involvement, including flaccid bullae and crusting. The lesions are frequently found in areas of repetitive trauma, such as the oral cavity and larynx. Histopathologic findings demonstrate acantholysis in the immediate suprabasilar layer of the epidermis, and direct immunofluorescence demonstrates intercellular staining of IgG and C3 throughout the stratified epithelium (Figure, B).1,2 The pathogenic IgG autoantibody is directed at the extracellular domain of desmogleins, which are cadherin proteins involved in cell-cell adhesion.2,3

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Article Information

Corresponding Author: Jeffrey Yu, MD, Department of Otolaryngology–Head and Neck Surgery, College of Medicine, University of Illinois at Chicago, 1855 W Taylor St, MC 648, Chicago, IL 60612 (jeffwyu@uic.edu).

Published Online: August 22, 2019. doi:10.1001/jamaoto.2019.2251

Conflict of Interest Disclosures: None reported.

Meeting Presentation: Information from this article was submitted and accepted for poster presentation at the American Academy of Otolaryngology–Head and Neck Surgery 2019 Annual Meeting & OTO Experience; September 15–18, 2019; New Orleans, Louisiana.

Additional Contributions: We would like to thank Marylee Braniecki, MD, for meeting and discussing the pathological findings, as well as providing our group with the images of the biopsies performed. She was not compensated for her contributions. We thank the patient for granting permission to publish this information.

References
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Mihai  S, Sitaru  C.  Immunopathology and molecular diagnosis of autoimmune bullous diseases.  J Cell Mol Med. 2007;11(3):462-481. PubMedGoogle ScholarCrossref
2.
Amagai  M, Koch  PJ, Nishikawa  T, Stanley  JR.  Pemphigus vulgaris antigen (desmoglein 3) is localized in the lower epidermis, the site of blister formation in patients.  J Invest Dermatol. 1996;106(2):351-355. doi:10.1111/1523-1747.ep12343081PubMedGoogle ScholarCrossref
3.
Amagai  M, Tsunoda  K, Zillikens  D, Nagai  T, Nishikawa  T.  The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile.  J Am Acad Dermatol. 1999;40(2):167-170.PubMedGoogle ScholarCrossref
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Marcoval  J, Servitje  O, Moreno  A, Jucglà  A, Peyrí  J.  Lupus vulgaris.  J Am Acad Dermatol. 1992;26(3):404-407. PubMedGoogle ScholarCrossref
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Fernández  S, España  A, Navedo  M, Barona  L.  Study of oral, ear, nose and throat involvement in pemphigus vulgaris by endoscopic examination.  Br J Dermatol. 2012;167(5):1011-1016. PubMedGoogle ScholarCrossref
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Bystryn  JC, Steinman  NM.  The adjuvant therapy of pemphigus.  Arch Dermatol. 1996;132(2):203-212. PubMedGoogle ScholarCrossref
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Murrell  DF, Peña  S, Joly  P,  et al.  Diagnosis and management of pemphigus: recommendations by an International Panel of Experts [published online February 10, 2018].  J Am Acad Dermatol. doi:10.1016/j.jaad.2018.02.021PubMedGoogle Scholar
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