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• W3149259628 abstract "Orofacial granulomatosis (OFG) presents with orofacial swelling, secondary to intra- and perilymphatic granuloma formation. Multiple etiologic associations have been proposed, but the pathogenesis remains unclear. One frequent association is Crohn's disease (CD) in pediatric patients. In this report, we present a case of an adult presenting with recurrent lip swelling, diagnosed with OFG, and later found to have severe CD in the absence of significant gastrointestinal symptoms. A 30-year old woman presented with intermittent swelling of the lower portion of her lip for 8 months. The swelling was painless and occasionally accompanied by clear fluid drainage. The upper portion of her lip later became involved. She reported symptomatic improvement with courses of oral corticosteroids. She had not applied any topical therapies and denied a history of seasonal allergies and cinnamon-flavored candy consumption. She denied associated stridor, shortness of breath, or any other mucosal changes. Review of systems revealed recent episodes of diarrhea and stiffness of her knees and wrists. Physical examination revealed rubbery edema of both lips with tiny vesicles (Fig 1). There was no appreciable facial paralysis or tongue changes, although a tongue piercing was in place. A biopsy was performed during her initial encounter, noting “proliferation of ectatic lymphatic channels in the lamina propria” (Fig 2). She found no improvement with doxycycline, azithromycin, topical corticosteroids, or intralesional triamcinolone. Patch testing revealed allergy to hydrocortisone. Laboratory studies, including complete blood count, antinuclear antibody, extractable nuclear antibodies, C1-esterase inhibitor, complements C1q and C4; chest x-ray, and rheumatoid factor were unremarkable. Her complete metabolic panel was significant for an albumin level of 2.5 g/dL.Fig 2The first biopsy specimen, revealing ectatic lymphatic channels in the lamina propria. (Hematoxylin-eosin stain.)View Large Image Figure ViewerDownload Hi-res image Download (PPT) A second biopsy 4 months later observed “non-necrotic granulomata in the lamina propria and edema and ectatic lymphatic vessels with intraluminal granuloma,” consistent with OFG with lymphangiectasia (Figs 3 and 4). A diagnosis of OFG was rendered. During dermatologic work-up, she presented to the emergency room for diarrhea. A computed tomography scan of the abdomen suggested colitis of inflammatory etiology. She was referred to gastroenterology, and endoscopy revealed ulcers of the entire esophagus, the ileum, the transverse, descending, and sigmoid colons; and anal canal, leading to a diagnosis of CD.Fig 4The second biopsy specimen, with focus on granulomata within lymphatic channels. (Hematoxylin-eosin stain.)View Large Image Figure ViewerDownload Hi-res image Download (PPT) Orofacial granulomatosis (OFG) most commonly presents with swelling of 1 or both lips,1Lazzerini M. Bramuzzo M. Ventura A. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.World J Gastroenterol. 2014; 20: 7497-7504Crossref PubMed Scopus (44) Google Scholar and has been observed uncommonly to present with lymphangiectasia.2Honigman A.D. Kim M. Chow C.W. Robertson S.J. Orofacial granulomatosis presenting with acquired lymphangiectasia.JAMA Dermatol. 2019; 155: 1320-1321Crossref PubMed Scopus (1) Google Scholar Oral mucosa and perioral skin may be involved, and some patients develop permanent swelling and disfigurement. Various pathologic factors have been proposed, including delayed hypersensitivity to dental materials or cinnamon, infections, oral dysbiosis3Al-Hamad A. Porter S. Fedele S. Orofacial granulomatosis.Dermatol Clin. 2015; 33: 433-446Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar and allergy.4Patel P. Brostoff J. Campbell H. et al.Clinical evidence for allergy in orofacial granulomatosis and inflammatory bowel disease.Clin Transl Allergy. 2013; 3: 26Crossref PubMed Scopus (30) Google Scholar There is a strong association between allergy and OFG, regardless of the presence of CD; however, it is not yet clear whether or not allergy is an etiopathologic factor in the development of OFG.4Patel P. Brostoff J. Campbell H. et al.Clinical evidence for allergy in orofacial granulomatosis and inflammatory bowel disease.Clin Transl Allergy. 2013; 3: 26Crossref PubMed Scopus (30) Google Scholar Dilated lymphatic channels and peri- or intralymphatic granuloma are seen on histopathology,5Marcoval J. Penin R.M. Histopathological features of orofacial granulomatosis.Am J Dermatopathol. 2016; 38: 194-200Crossref PubMed Scopus (20) Google Scholar as were observed in our second biopsy. It is worth noting that the pinpoint vesicles observed initially reflected early lymphangiectatic changes and were the presenting sign of early intra- and perilymphatic granuloma aggregation. Serial biopsy allowed for temporal observation of the progressive lymphatic changes that would occur in OFG. There is a strong association between OFG and CD in pediatric patients; approximately 50% of those diagnosed with OFG will develop CD, at a mean lag time of 13 months,1Lazzerini M. Bramuzzo M. Ventura A. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.World J Gastroenterol. 2014; 20: 7497-7504Crossref PubMed Scopus (44) Google Scholar and pediatric CD-OFG patients are more likely to have pan-enteric disease with extensive intestinal granuloma formation.6Gale G. Sigurdsson G.V. Östman S. et al.Does Crohn's disease with concomitant orofacial granulomatosis represent a distinctive disease subtype?.Inflamm Bowel Dis. 2016; 22: 1071-1077Crossref PubMed Scopus (16) Google Scholar The association between OFG and CD in adult patients is not well defined, and OFG does not necessarily precede the onset of CD,1Lazzerini M. Bramuzzo M. Ventura A. Association between orofacial granulomatosis and Crohn's disease in children: systematic review.World J Gastroenterol. 2014; 20: 7497-7504Crossref PubMed Scopus (44) Google Scholar,7Campbell H. Escudier M. Patel P. et al.Distinguishing orofacial granulomatosis from Crohn's disease: two separate disease entities?.Inflamm Bowel Dis. 2011; 17: 2109-2115Crossref PubMed Scopus (78) Google Scholar with only 20% eventually developing CD. Although judicious surveillance of pediatric OFG patients for systemic symptoms is essential, this case report serves to highlight that the OFG-CD association may also be observed in adults. Treatment of OFG is difficult, attributed to inadequate appreciation of the etiopathogenesis.8Banks T. Gada S. A comprehensive review of current treatments for granulomatous cheilitis.Br J Dermatol. 2012; 166: 934-937Crossref PubMed Scopus (53) Google Scholar There are reports of successful treatment with topical, intralesional, and systemic corticosteroid use, although no individual treatment has eventuated as superior. Surgical management via cheiloplasty is a last resort.8Banks T. Gada S. A comprehensive review of current treatments for granulomatous cheilitis.Br J Dermatol. 2012; 166: 934-937Crossref PubMed Scopus (53) Google Scholar Recent reports have demonstrated efficacy of tumor necrosis factor-alpha antagonist use.9O'Neill I.D. Scully C. Biologics in oral medicine: oral Crohn's disease and orofacial granulomatosis.Oral Dis. 2012; 18: 633-638Crossref PubMed Scopus (23) Google Scholar Successful tandem treatment of OFG and CD with infliximab has been reported in case series, likely owing to the shared pathogenesis in these patients.9O'Neill I.D. Scully C. Biologics in oral medicine: oral Crohn's disease and orofacial granulomatosis.Oral Dis. 2012; 18: 633-638Crossref PubMed Scopus (23) Google Scholar The present patient started treatment with methotrexate and infliximab per gastroenterology and noted rapid improvement in her OFG, as well as increased energy levels and resolution of diarrhea and arthralgia. At follow-up, there was significant improvement in lip swelling, induration, and rubbery texture. No further vesicles have been observed. It is important to include OFG in the differential diagnosis of patients with lip swelling and to screen for inflammatory bowel disease, as OFG may hallmark severe CD in the absence of significant gastrointestinal symptoms in adults as well as children. None declared." @default.
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• W3149259628 title "Lip swelling with lymphangiectasia" @default.
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