APPROACHING THE STIFF UPPER LIP: A CASE OF PSEUDOANGIOEDEMA DIAGNOSED AS OROFACIAL GRANULOMATOSIS

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<h3>Introduction</h3> Angioedema describes transient swelling in the deeper layers of the skin and mucosa, broadly categorized as histaminergic or bradykinin-mediated. Allergists should also be familiar with conditions that mimic angioedema to hasten proper management and avoid negative impact from misdiagnosis. We describe a case of one such condition, Orofacial granulomatosis with pyostomatitis vegetans, involving disfiguring inflammation of the orofacial soft tissue and often associated with comorbid inflammatory bowel disease. <h3>Case Description</h3> A 61-year-old female with a history of dysphagia, COPD, Esophageal stenosis and stroke was referred to Allergy for ten years of recurrent facial and lip swelling. ER visits resulted in multiple rounds of corticosteroids with variable success. Antibiotics for presumed cellulitis, epinephrine, high dose antihistamines and bradykinin antagonists (icatibant) were unsuccessful. Work up included normal complement studies (C3, C4, C1q, C1 esterase quantity/function). After being lost to follow up, the patient re-presented with firm, fixed lip swelling (figure 1), pustules of the buccal mucosa, dysphagia and diarrhea, prompting hospital admission. Buccal biopsy demonstrated pathology suggestive of pyostomatitis vegetans, a rare inflammatory dermatosis strongly associated with IBD. Labs demonstrated high titer positivity for <i>saccharomyces cerevisiae</i> antibody (ASCA) IgG and IgA, with a 95% specificity for IBD, particularly ulcerative colitis. She is currently being trialed on empiric anti-TNF therapy with adalimumab. <h3>Discussion</h3> This is a rare case of pseudoangioedema from Orofacial granulomatosis with pyostomatitis vegetans in the setting of suspected inflammatory bowel disease. The progressive nature and complexity of this disease underscore the importance of Allergists being familiar with disorders that mimic angioedema.