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Home , Lichen planus, Orofacial granulomatosis

CASE REPORT

New-onset oral planus and granulomatous in a 66-year-old woman

Annie Ferguson, MD, Spring Golden, MD, and Lynne Morrison, MD Portland, Oregon Key words: Crohn’s disease; inflammatory condition; Melkersson-Rosenthal syndrome; Miescher’s granulomatous cheilitis; oral ; ; oral .

INTRODUCTION Abbreviations used: We report a case of a 66-year-old woman with the simultaneous onset of oral lichen planus (OLP) and GC: granulomatous cheilitis OFG: orofacial granulomatosis granulomatous cheilitis (GC). Both diseases are OLCR: oral lichenoid contact reaction relatively rare, and further investigation for systemic OLP: oral lichen planus or environmental causes of disease is warranted in OLTR: oral lichenoid tissue reactions new patients with either OLP or GC. This case and reports of a so-called lichenoid and granulomatous in the literature beg the question, is there overlying mild epithelial some unifying etiologic explanation for OLP and GC?1 with spongiosis and a superficial perivascular to nodular infiltrate of , plasma cells, and CASE REPORT histiocytes (Fig 4). Stains for fungal and acid-fast A 66-year-old woman presented with 1 year of organisms were negative, and polarized microscopy unremitting upper swelling and anterior gingival did not show foreign material. The histopathology of pain, made worse by spicy or citrus foods. Her the upper and lower lip was consistent with GC and history was negative for new products OLP, respectively. or foods in her diet, and she had no known . She had undergone extensive dental work during the DISCUSSION last 2 years. Physical examination found of In 1945, the term granulomatous cheilitis was the upper lip and reticulated plaques surrounded by used to label granulomatous infiltration and on the lower lip and the left lateral tongue lymphatic obstruction of the . Decades earlier, (Figs 1 and 2). The upper anterior gingiva had a the neuromucocutaneous triad of orofacial edema, beefy red, well-demarcated erythematous appear- tongue fissuring, and unilateral facial paralysis, ance (Fig 3). A complete blood count, comprehen- indistinguishable from Bell’s palsy, was identified sive metabolic panel, and chest radiograph were by Melkersson and Rosenthal.2 In 1985, the term within normal limits. Her history was negative for orofacial granulomatosis (OFG) was coined, and gastrointestinal symptoms, and she had a normal referred to noncaseating of the face and screening colonoscopy 2 years prior. Microscopic oral cavity in the absence of systemic granulomatous examination of a 3- 3 2- 3 2-mm shave disease. OFG encompasses a spectrum of cutaneous section of the lower lip found a superficial bandlike, granulomatous conditions from Miescher’s GC to inflammatory infiltrate focally obscuring the dermo- Melkersson-Rosenthal syndrome. OFG is a diagnosis epidermal junction. Direct immunofluorescence of exclusion, as localized granulomatous swelling of showed irregular deposition of fibrinogen along the oral cavity may be caused by systemic disease. the and no specific deposition Therefore, perioral granulomas warrant further of IgG, IgA, IgM or C3. A 9- 3 5- 3 3-mm incisional workup to rule out conditions including Crohn’s biopsy of the upper vermillion border showed disease, and . Isolated

From the Department, Oregon Health & Science 2352-5126 University. Ó 2016 by the American Academy of Dermatology, Inc. Published Funding sources: None. by Elsevier, Inc. This is an open access article under the CC BY- Conflicts of interest: None declared. NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ Correspondence to: Annie Ferguson, MD, Mail code: CH16D, 3303 SW 4.0/). Bond Ave, Portland, OR 97239-4501. E-mail: [email protected]. http://dx.doi.org/10.1016/j.jdcr.2016.02.006 JAAD Case Reports 2016;2:177-80.

177 178 Ferguson, Golden, and Morrison JAAD CASE REPORTS MARCH 2016

Fig 1. A and B, Upper lip swelling associated with granulomatous cheilitis.

Fig 2. Oral lichen planus presenting as on the lateral tongue (A) and (B).

requiring reconstructive plastic surgery in extreme cases. The etiopathogenesis is not definitively under- stood, although it is postulated to be a delayed-type reaction.5,6 Foods, flavorings, addi- tives, dental materials, and infectious agents have all been implicated. Case reports describe remission with dietary restriction. However, no rigorous in vitro or in vivo studies have been conducted to validate immunologic or inflammatory origins of OFG.5,6 A combination of systemic and Fig 3. Edematous- and erythematous-appearing upper minocycline is considered the best therapeutic gingiva. regimen. There is considerable histopathologic mimicry among granulomatous conditions. cutaneous granulomas may also occur as a foreign OFG and oral Crohn’s disease are histologically body reaction. identical, and some investigators conjecture that is the most common sign of OFG, but OFG is a manifestation of Crohn’s disease rather oral ulcers, vertical fissures of the lips, mucosal tags, than an independent entity. Many OFG cases are cobblestoned oral mucosa, , and cervical rediagnosed as Crohn’s disease because of the involvement have been described.3,4 eventual onset of intestinal symptoms.7 Although Melkersson-Rosenthal syndrome can also present not all patients with OFG go on to have full-blown with facial swelling, unilateral facial palsy, fissured Crohn’s disease, this does not exclude the possibility tongue, and dysgeusia. that all cases of OFG are manifestations of Crohn’s Clinically, GC presents with painless swelling of disease limited to the face and mouth. Debate the lip, which may occur once and resolve but more continues, but patients with OFG require prolonged commonly evolves into a chronic condition. Swelling follow-up to evaluate for evolution into Crohn’s is initially soft, but after several attacks becomes disease. indurated. Fibrosis can cause permanent disfigure- OLP is an inflammatory condition, ranging from ment, leading to problems with speech and eating, asymptomatic to severe, painful ulcers. OLP JAAD CASE REPORTS Ferguson, Golden, and Morrison 179 VOLUME 2, NUMBER 2

Fig 4. A-C, Biopsy section from the tongue shows irregular epithelial hyperplasia, basal layer vacuolar alteration, and lichenoid with scattered necrotic , typical of oral lichen planus. (Original magnifications: A, 310; B, 320; and C, 340.) usually presents in middle age with slight female contact reactions (OLCR) likely represent a delayed predominance and can affect the buccal mucosa, hypersensitivity reaction and removal of the allergen gingiva, tongue, and vermillion border of the lip. can lead to complete resolution.10 Given the Idiopathic OLP is believed to be a T- cellemediated similarities, oral lichenoid contact reactions can be response toward an unknown and may misdiagnosed as OLP, leading to improper disease represent an autoimmune reaction. Spontaneous management. This patient presented with a rare and remission is rare, and therapy is directed toward unique set of findings. The simultaneous onset of dampening the inflammatory response using OLP and GC in an otherwise healthy woman may corticosteroids or immune modulators. Patients very well indicate a common immunologic or may be refractory to treatment, and although inflammatory etiology for 2 uncommon diseases of reduction in symptoms can be achieved, no the oral cavity. definitive cure has been identified. OLP carries a significant risk for transformation into squamous cell REFERENCES carcinoma, requiring surveillance for malignant 1. Robinson CM, Oxley JD, Weir J, Eveson JW. Lichenoid changes.8 On examination, classic OLP is often and granulomatous : an entity or a non- indistinguishable from oral lichenoid tissue reactions specific inflammatory process? J Oral Pathol Med. 2006;35: 262-267. (OLTR), highlighting the importance of thorough 2. Rogers R. Granulomatous Cheilitis, Melkersson-Rosenthal history taking. OLTR may be caused by systemic Syndrome, and Orofacial Granulomatosis. Arch Dermatol. drug effects, localized contact reactions, or 2000;136:1157-1158. graft-versus-host disease.9 Adding to the diagnostic 3. Mignogna M, Fedele S, Lo Russo L, Lo Muzio L. Orofacial challenge, idiopathic OLP and OLTR have an iden- granulomatosis with gingival onset. J Clin Periodontol. 2001;28: 692-696. tical histologic appearance that includes liquefactive 4. Grave B, McCullough M, Wiesenfeld D. Orofacial - changes of the basal keratinocytes with a bandlike tosis—a 20-year review. Oral Diseases. 2009;15:46-51. array of activated T lymphocytes, macrophages, and 5. McCartan BE, Healy CM, McCreary CE, Flint SR, Rogers S, dendritic cells. Histology isn’t the diagnostic Toner ME. Characteristics of patients with orofacial gold standard for OLP, and many infectious and granulomatosis. Oral Diseases. 2011;17:696-704. 6. Tilakaratne WM, Freysdottir J, Fortune F. Orofacial granuloma- immune-mediated diseases can induce oral tosis: review on aetiology and pathogenesis. J Oral Pathol Med. lichenoid changes that mimic OLP. Oral lichenoid 2008;37:191-195. 180 Ferguson, Golden, and Morrison JAAD CASE REPORTS MARCH 2016

7. Carr D. Granulomatous cheilitis in Crohn’s disease. Br Med J. 9. Khudhur AS, Di Zenzo G, Carrozzo M. Oral lichenoid tissue 1974;4:636. reactions: diagnosis and classification. Expert Rev Mol Diagn. 8. Gandolfo S, Richiardi L, Carrozzo M. Risk of oral squamous 2014;14:169-184. cell carcinoma in 402 patients with oral lichen planus: a 10. Yiannias JA, el-Azhary R, Hand JH, Pakzad SY, Rogers RS. follow-up study in an Italian population. Oral Oncol. 2004;40: Relevant contact sensitivities in patients with the diagnosis of 77-83. oral lichen planus. J Am Acad Dermatol. 2000;42:177-182.