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Linear IgA dermatosis limited to the mucosal membranes as a rare cause of dysphagia.

Linear immunoglobulin A (IgA) bullous dermatosis (LAD) is a rare cutaneous disease resulting in subepidermal blisters. Clinically, it is difficult to distinguish linear IgA bullous dermatosis from other chronic blistering disorders. It is characterized by IgA antibodies at the basement membrane zone (IgA anti-BMZ) deposited in a linear manner. (1) The gold standard for diagnosis is linear IgA anti-BMZ deposits on direct immunofluorescence. IgG, IgM, and complement component 3 (C3) are typically absent in LAD. (2)

As many as 50 to 70% of patients with LAD exhibit involvement of the mucous membranes (3)--typically oral cavity and conjunctival lesions. However, few cases with solely mucous membrane involvement have been reported. (2,4-6) Here we report a case of linear IgA dermatosis limited to the pharynx, resulting in symptomatic dysphagia and odynophagia.

Our patient was a 68-year-old Caucasian woman with systemic lupus erythematosus and polymyositis who presented with a gradual onset of dysphagia and odynophagia. Physical examination was notable for erythematous oral mucosa with desquamating lesions of the posterior pharyngeal wall that were temporarily responsive to steroid treatment. A computed tomography scan of her neck with contrast was negative for suspicious lesions, and she had no exam findings concerning for malignancy.

On biopsy, the patient's hematoxylin-eosin stained sections revealed subepithelial detachment of the squamous mucosa with associated neutrophilic infiltrate. Direct immunofluorescence (DIF) was consistent with a subepithelial bullous disease. DIF studies demonstrated linear IgA (4+) staining in the basement membrane zone. There was no significant staining seen with IgG, IgM, C3, fibrin, or albumin. These findings are consistent with the published description of linear IgA disease.

During evaluation of the patient, the erythematous lesions of the oropharynx were noted on physical exam. Flexible fiberoptic laryngoscopy revealed a normal nasal cavity and nasopharyngeal mucosa. The desquamating lesions on her posterior pharyngeal wall extended to the bilateral arytenoids, posterior cricoid region, and piriform sinuses, as well as the subglottic region (figure).

Linear IgA dermatosis is a rare autoimmune subepidermal bullous disease that is rarely encountered by the otolaryngologist. This case highlights the need for practicing otolaryngologists to be aware of this disease and its possible manifestation in the head and neck mucosa. In the unusual situation in which a patient presents without cutaneous lesions, such as the case we presented here, it is the responsibility of the otolaryngologist to consider this entity as a possibility and confirm the diagnosis with tissue biopsy.

References

(1.) James WD, Berger TG, Elston DM. Chronic blistering dermatoses. In: James WD (ed). Andrews' Diseases of the Skin: Clinical Dermatology. 12th ed. Philadelphia: Elsevier; 2015:45170.

(2.) Dan H, Lu R, Li W, et al. Linear IgA disease limited to the oral mucosa. J Am Acad Dermatol 2011;65(3):677-9.

(3.) Guide SV, Marinkovich MP. Linear IgA bullous dermatosis. Clin Dermatol 2001;19(6):719-27.

(4.) Gluth MB, Witman PM, Thompson DM. Upper aerodigestive tract complications in a neonate with linear IgA bullous dermatosis. Int J Pediatr Otorhinolaryngol 2004;68(7):965-70.

(5.) Equia del Valle A, Aguirre Urizar JM, Martinez Sahuguillo A. Oral manifestations caused by the linear IgA disease. Med Oral 2004;9(1):39-44.

(6.) Joseph TI, Sathyan P, Goma Kumar KU. Linear IgA dermatosis adult variant with oral manifestation: A rare caseaaaa report. J Oral Maxillofac Pathol 2015;19(1):83-7.

Victoria M. Bones, MD; Lyndsay L. Madden, DO

From the Department of Otolaryngology-Head and Neck Surgery, Wake Forest School of Medicine, Winston-Salem, N.C.

Caption: Figure. Flexible fiberoptic laryngoscopy demonstrates the desquamating lesions of the subglottic region (arrow) (A) and the posterior cricoid, aryepiglottic folds, and piriform sinuses (arrows) (B).
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Title Annotation:DYSPHAGIA CLINIC
Author:Bones, Victoria M.; Madden, Lyndsay L.
Publication:Ear, Nose and Throat Journal
Article Type:Report
Date:Apr 1, 2017
Words:597
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