Abstract
Diagnosis of oral ulceration is always challenging for clinicians due to remarkable overlap in their clinical presentations. Most ulcerative lesions of the oral mucosa fall into one of four categories: infection, immune related, traumatic, or neoplastic. Behcet’s disease is a systemic inflammatory disease of unknown etiology which can be presented with recurrent oral and genital ulcers. The disease is strongly associated with human leukocyte antigen (HLA B51). 1 The treatment depends on severity of the illness and requires a multidisciplinary approach. This case report summarizes a case presenting a diagnostic dilemma as it was initially thought to be oral mycotic ulcer but later on it turned out to be a case of Behçet’s disease.
Introduction
Ulcerated lesions of the oral cavity have many underlying etiologic factors, most commonly infection, immune related, traumatic, or neoplastic. A detailed evaluation in history is critical in assessing ulcerative oral lesions and should include any inciting or triggering trauma, medical conditions, medication history, the length of time the lesion has been present; the frequency of episodes in recurrent cases; the presence or absence of pain; and the growth of the lesion over time. For multiple or recurrent lesions, the presence or history of ulcers on the skin, genital areas, or eyes should be evaluated along with any accompanying systemic symptoms such as fever, arthritis, or other signs of underlying systemic disease. Biopsy may be indicated in many ulcerative lesions of the oral cavity although some are more suitable for clinical diagnosis. Adjunctive tests may be required for final diagnosis of some ulcerated lesions especially autoimmune lesions. Laboratory tests to rule out systemic disease may be also required for recurrent or severe ulcerations especially when accompanied by other symptoms. 1
Case report
43 yr old man chronic smoker, betel chewer from Rakhine state was referred to Department of Tropical and Infectious Diseases, YGH from ENT hospital with oral ulcer for 7 months and fever for 5 days. Regarding oral ulcer, it gradually increased in size at hard palate over 1 month period, associated with pain and swallowing difficulty but no blood and pus discharge from the ulcer. He had history of similar problem last 5 months ago which was relieved by some local treatment only. Fever was high grade and associated with oral pain. Apart from that, there was no history of preceding trauma, joint pain, genital ulcers and vision problem. No positive symptoms were detected on system review. He had no history of hypertension, diabetes mellitus, renal and liver diseases and not taking immunosuppressants. On examination patient was afebrile but vital signs were stable. On examination of oral cavity, there were 3 oral ulcers over the hard palate area and inner aspects of the lower lip, 3×4 cm in greatest dimension with few pus flakes. (picture A) No dental caries and cervical lymph node enlargement. No abnormalities were detected in systemic examinations. Before admission to YGH, he consulted to ENT surgeon at ENT hospital for oral ulcer. The biopsy was from the ulcer which revealed that active chronic ulcer with fungal infection (Probably Candidiasis) (PAS+). His routine investigations revealed that except mild normocytic normochromic anaemia and high ESR 115 and CRP 98, liver, renal function were within normal limits. Hepatitis B, C, VDRL and HIV status were negative. ANA 1/100. Blood culture was sterile. Sputum fungal culture detected that mixed growth of Aspergillus fumigatus and Candida albican. CXR (PA), USG (abd), Echocardiogram stated no abnormality. He was treated with IV antibiotics and IV amphotericin according to sensitivity result. After treatment, the fever subsided and pain was relieved but oral ulcer was still present. Since this was recurrent oral ulcer and not fully responded to antifungal treatment, pathergy tests was done after consultation with Rheumatologist and it turned out to be positive. According to international criteria for Behçet’s disease, it was fulfilled criteria for the possible case. (Table 1). As per suggestion from rheumatologist, PO colchicine and IV methyl prednisone were started. His oral ulcer was dramatically improved after treatment (picture B). He was planned to take joint care with our team (infectious disease physicians) and rheumatologist for further management and follow up.
Picture (A) oral ulcer on arrival
Picture(B) oral ulcer after treatment
Table (1) Classification criteria for Behcet’s Syndrome
Discussion
Because of the variety of presenting features and causative factors, identification of oral ulcerative lesions may be relatively challenging. Local or systemic factors can be contributing to developing ulcers 2,3. Ulcers have different parts: the floor (uncovered ulcer surface), the base (ulcer rest seat), the margin (interface among the wall of ulcer and normal epithelium) and the edge (the part of the margin and floor). The extension phase, transition phase (preparation for healing) and the healing or repair phase are the three stages that are identified throughout a simple ulcer clinical course 4. The oral cavity provides a unique ecological site that harbors a dynamic microbiota. Candida, Aspergillus, and Rhizopus are commensal oral fungi species. However, they may become pathological and cause opportunistic infections under certain conditions. The incidence of fungal infections has been on the rise in recent years, especially in COVID-19 patients, due to several factors such as immunodeficiency, hyperglycemia caused by diabetes mellitus, prolonged hospitalizations, and prolonged use of steroids, and hematopoietic malignancies. 5,6 The diagnosis of oral fungal infection is mainly based on clinical presentation and histopathology of the lesioned tissue. Characteristic features include a burning sensation and an unpleasant taste in the mouth, white plaques, ulcerations, erythema, and pain 6 Clinical assessment of oral lesions, recognizing and correcting the underlying predisposing factors, and administering appropriate antifungal agents are the cornerstones for managing oral mycoses. Behcet disease is an auto-inflammatory systemic vasculitis of unknown etiology. It is characterized by mucocutaneous manifestations, including recurrent oral and genital ulcerations, ocular manifestations, especially chronic relapsing uveitis, and systemic vasculitis involving arteries and veins of all sizes. Oral ulcers occur in 97% to 99% of patients with Behcet disease, often representing the initial clinical feature. Lesions are usually painful, recurrent, and multiple and may involve the soft palate, hard palate, buccal mucosa, tongue, gingiva, lips, and tonsils. 7They’re often the first noticed symptom and may occur 2–3years before any other symptoms of Behcet disease of appear.8 Many treatments have been used to decrease morbidity and mortality. Corticosteroids can help reduce the inflammation associated with Behçet’s disease. First-line treatment for non-severe manifestations include topical anti-inflammatory therapy and the
use of colchicine to prevent recurrence oral ulcer. For major organ manifestations and refractory conditions glucocorticoids and synthetic or biologic immunosuppressive agents can be used. 9 In our case, although he was not fulfilled all 4 criteria for Behcet disease, his oral ulcer was rapidly responded to steroid and partially responded to anti-fungal treatment. Therefore, it was regarded as Behcet disease and continued treatment to prevent later complications of Behcet disease.
Conclusion
Prompt evaluation of oral lesions is essential to establish the diagnosis, initiate therapy, and monitor clinical progress. We report a case of Behcet’ disease presented initially with recurrent oral ulcer with superadded Aspergillus and Candida infection. Awareness of this rare condition should be thought of by healthcare professionals.
Reference
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Author Information
Aye-Mya-Theingi-Win1, Khin-Rupar-Ko2, Aye-Aye-Win3, May-Zabe4, Nyunt Thein5
1. Consultant physician, Tropical and Infectious Diseases Department, Yangon General Hospital
2. Professor, Tropical and Infectious Diseases Department, Yangon General Hospital
3. Associated Professor, Tropical and Infectious Diseases Department, University of Medicine (1), Yangon
4. Consultant physician, Tropical and Infectious Diseases Department, Yangon General Hospital
5. Senior Consultant physician, Former Head of Department of Medicine, Emeritus Professor of Medicine, University of Medicine (1), Yangon
