Lip Swelling Research Articles

SAVED BY THE BAL: A CASE OF ACUTE EOSINOPHILIC PNEUMONIA AFTER METHYL-NALTREXONE INJECTION

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Eosinophilic pneumonia (EP) can result as an adverse effect of many drugs. Methyl-naltrexone injection has become increasingly popular this case serves to bring attention to a rare, but potential adverse effects. CASE PRESENTATION: A 44-year-old male with history of alcohol use disorder was brought to the emergency department (ED) by ambulance in acute respiratory failure. He received his monthly naltrexone injection three days prior. The following day, he started having lip swelling, rash, and two days later, had dyspnea. Upon arrival to the ED, he was given two doses of IM epinephrine, as well as methylprednisolone 125 mg IV, and he required BiPAP, after what was thought to be an anaphylactic reaction to naltrexone. Chest x-ray revealed stable bilateral lower lobe opacities, possibly related to aspiration pneumonia, patient was placed on ampicillin-sulbactam. CBC demonstrated increased absolute levels of eosinophils (700k/mm3), anaphylaxis was ruled out with tryptase level of 2.8 (normal <11.5). Patient’s symptoms slightly improved, however patient's eosinophil count continued to trend upwards, as high as 2500k/mm3 four days after presentation. CTA of chest demonstrated no evidence of pulmonary embolism, but did reveal diffuse ground glass opacities consistent with acute infiltrates. Bronchoscopy with bronchoalveolar lavage (BAL) was performed and BAL yielded fluid with eosinophils making up greater than 50% of the WBC's and the fluid was negative for malignant cells. Eosinophilic pneumonia diagnosis was established and steroids were initiated with improvement of symptoms. DISCUSSION: Acute eosinophic pneumonia is characterized by acute onset febrile illness, hypoxemia, diffuse bilateral infiltrates on imaging, and pulmonary eosinophilia with more than 25% eosinophils in BAL. EP is characterized by symptoms lasting less than 1 month and usually less than 1 week. EP will respond rapidly to steroid therapy but early recognition remains important to choose appropriate therapy given mimics such as infection. Several medications have historically been shown to cause EP, in one review of the literature the most commonly cited drugs include antibiotics or anti-inflammatories. Imaging findings typically include bilateral reticular ground-glass opacities that expand as the disease progresses. Methyl-naltrexone is increasingly utilized as an effective treatment for alcohol dependence disorder. Its possible association with EP was initially noted in a large randomized control trial published in JAMA by Garbutt et al. In our review of the literature there have been only four other cases of naltrexone induced eosinophilic pneumonia. CONCLUSIONS: This case demonstrates a likely outcome of eosinophilic pneumonia in a patient receiving methyl-naltrexone injections for alcohol use disorder. It is important to recognize naltrexone as a possible etiology for eosinophilic pneumonia due to its increasing use. Reference #1: Carmi Bartal, MD, MHA, Iftach Sagy, MD, and Leonid Barski, MD. Medicine (Baltimore). 2018 Jan; 97(4): e9688. Published online 2018 Jan 26. Drug-induced eosinophilic pneumonia: A review of 196 case reports Reference #2: Kim H, Ali M, Buch K. Eosinophilic Pneumonia Induced by Injectable Naltrexone. Am J Respir Crit Care Med. 2014;189:A2283 Reference #3: Garbutt JC, Kranzler HR, O'Malley SS, Gastfriend DR, Pettinati HM, Silverman BL, Loewy JW, Ehrich EW; Vivitrex Study Group. Efficacy and tolerability of long-acting injectable naltrexone for alcohol dependence: a randomized controlled trial. JAMA. 2005 Apr 6;293(13):1617-25 DISCLOSURES: No relevant relationships by Anthony Esposito, source=Web Response No relevant relationships by benison lau, source=Web Response

2504 Successful Budesonide Monotherapy for Autoimmune Hepatitis/Hepatic Sarcoidosis Overlap Syndrome in a Patient With Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) From Azathioprine

INTRODUCTION: Drug reaction with eosinophilia and systemic symptoms (DRESS) is considered a severe idiosyncratic drug hypersensitivity which can be fatal if not promptly diagnosed and treated. Here, we describe a case of hepatic sarcoidosis and autoimmune hepatitis (AIH) overlap syndrome who was found to have DRESS from taking azathioprine (AZA). CASE DESCRIPTION/METHODS: A 63-year-old African-American female with a diagnosis of AIH and hepatic sarcoidosis overlap based on liver biopsy (chronic hepatitis with moderate activity (grade 2/4), mild fibrosis (stage 1/4), non-caseating granulomata, and interface hepatitis with dense lymphoplasmacytic infiltration) (Figure 1), positive ANA (1:160), and elevated IgG (2140) was started on treatment with AZA and budesonide (intolerance to prednisone). She had normal TPMT activity. Patient stopped taking AZA few days later because of feeling “jittery.” She presented to the ED five weeks later with lip swelling and desquamating rash and was discharged with antihistamines. She returned to the ED two days later with worsening rash, fever, and dyspnea. Labs showed leukocytosis, eosinophilia (56.1%, 13,400/mm3), acute hepatitis, acute kidney injury, and respiratory distress with concern for DRESS. Her initial LFTs were ALT 96, AST 65, and Alk Phos 633, which were increased from her baseline. Imaging ruled out biliary obstruction and bone marrow biopsy was negative for myeloproliferative disorders such as hypereosinophilic syndrome. She was diagnosed with DRESS secondary to AZA and was treated with a short course of prednisone with transition to budesonide monotherapy. Eosinophilia and other organ dysfunction resolved. Her most recent LFTs are ALT 37, AST 42, and Alk Phos 277. DISCUSSION: While AZA hypersensitivity syndrome is a well-known disorder presenting with drug eruption and flu-like symptoms, to our knowledge this is the first case of DRESS secondary to AZA. Additionally, this occurred in the setting of hepatic sarcoidosis and AIH overlap syndrome which itself is a rare entity. DRESS is characterized by a long latency time between exposure and disease (2-8 weeks) and presents with end organ dysfunction requiring steroid treatment. Based on the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system, our patient's score was 7, where a score of 6 or higher is considered definite for DRESS. This case also stresses the value of budesonide monotherapy for treating AIH and hepatic sarcoidosis overlap when other first line medications cannot be used.

A RARE CASE OF PARANEOPLASTIC EDEMATOUS DERMATOMYOSITIS ASSOCIATED WITH SMALL CELL LUNG CANCER

SESSION TITLE: Lung Cancer: Unusual Presentations SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Paraneoplastic edematous dermatomyositis (EDM) as a manifestation of Small Cell Lung Cancer (SCLC) is a rare process with one reported case in the literature. CASE PRESENTATION: We present a 51-year-old, previously healthy woman with a nine pack-year smoking history, who presented with a one-month history of progressive facial edema, facial erythema, lip swelling, and a violaceous atypical rash with crusting lesions involving her extremities, anterior neck, chest wall (V sign), and periungual area. The erythematous rash with blistering initially developed on the undersurface of both forearms after exposure to a wheelchair which the patient attributed to a nickel allergy. The patient failed low-dose outpatient steroids and was admitted with progressive lip swelling and facial edema. Over the course of her hospitalization, the patient developed significant proximal muscle weakness, proximal muscle tenderness, and was found to have an elevated creatine kinase level of 5,912 [IU]/L, antinuclear antibody titer was positive at 1:640 dilution, and a dermal biopsy consistent with dermatomyositis. Chest CT revealed a hilar mass with precarinal adenopathy. The patient underwent endobronchial ultrasound with transbronchial aspirate demonstrating SCLC. The patient’s facial edema and atypical rash responded to high-dose oral prednisone although proximal weakness persisted at discharge from the hospital. Upon follow up with hematology and oncology, the patient was formally diagnosed with limited stage SCLC and initiated on etoposide and carboplatin with radiation therapy. Additional evaluation with CT Abdomen demonstrated no evidence of distant metastasis. After completion of her chemoradiotherapy, her subcutaneous edematous rash resolved. DISCUSSION: A review of the literature suggests that EDM is rare with only 23 cases reported to date. EDM may be associated with a higher mortality and a more fulminant course. CONCLUSIONS: Familiarity with this paraneoplastic phenomenon may lead to early diagnosis of treatable malignancies and decrease short term mortality. Reference #1: Zarrabi, K., et al., Paraneoplastic edematous dermatomyositis: A rare syndromeobserved in a case of small cell lung cancer. Clinics and practice, 2017. 7(4). Reference #2: Milisenda JC, Doti PI, Prieto-González S, Grau JM. Dermatomyositis presenting withsevere subcutaneous edema: five additional cases and review of the literature. SeminArthritis Rheum 2014; 44:228. DISCLOSURES: No relevant relationships by James Davidson, source=Web Response No relevant relationships by Jeremy Harvey, source=Web Response No relevant relationships by Rahul Sampath, source=Web Response No relevant relationships by Mathew Thomas, source=Web Response No relevant relationships by Ly Tran, source=Web Response

Cancrum Oris(Noma): An Early Sign of Acute Lymphoblastic Leukemia Relapse

Abstract Cancrum oris is an infectious disease, which involves the orofacial tissues and adjacent neighboring structures in its fulminant course. Cancrum oris can occur rarely in chemotherapy-induced neutropenic patients with acute lymphoblastic leukemia. This rare case report reveals that cancrum oris can be a sign of acute lymphoblastic leukemia relapse. It is characterized by gangrenous lesions on skin with fine-needle aspiration cytology smear from the lesion showing infiltration of lymphoblasts. Cancrum oris can occur rarely in chemotherapy-induced neutropenic patients with acute lymphoblastic leukemia, but the presentation as an early sign of extramedullary disease relapse has not been documented in literature. Here we report a case of cancrum oris in a 27-year-old woman who presented with redness and swelling of the upper lip and infranasal area. She had a known case of acute lymphoblastic leukemia diagnosed 6 months back and was on regular multiagent chemotherapy for the same. Clinical examination revealed gangrenous lesions on infranasal, intranasal, and area involving the upper lip. Complete blood count, bone marrow aspiration, and cytology did not show any lymphoblasts. Blood culture and swab culture from the gangrenous area were sterile. Patient did not get better even after 7 days of broad-spectrum antibiotic treatment. With a high degree of clinical suspicion of isolated extramedullary relapse, fine-needle aspiration cytology from marginal and gangrenous area was taken and revealed infiltration of soft tissues by lymphoblasts. After receiving four cycles of multiple chemotherapy drugs, the skin lesions resolved. Treatment of cancrum oris involves the correction of the underlying immune status, antibiotics, and surgical reconstruction. The rare possibility of relapse of acute lymphoblastic leukemia presenting as cancrum oris should be kept in mind in patients with acute lymphoblastic leukemia on chemotherapy with skin manifestations.

11. An unusual case of dermatomyositis with anti-small ubiquitin-like modifier activating enzyme (SAE) antibodies present

IntroductionIdiopathic inflammatory myopathies (IIM) have long been diagnosed using a defined number of clinical criteria (Bohan and Peter). The emergence of new myositis specific antibodies (MSAs) and their relation to specific disease phenotypes may be useful in establishing a new clinical-serological diagnostic criteria for different disease presentations and thus help to determine management and prognosis. We present a case of dermatomyositis (DM) where limb subcutaneous oedema; a rare manifestation of the disease, and severe dysphagia were prominent clinical features in addition with the presence of anti-small ubiquitin-like modifier activating enzyme (SAE) antibodies.Case descriptionA 63-year-old Pakistani male presented with weight loss, anorexia, odynophagia, and a rash over his scalp, chest, face and flexor surfaces. Initial blood results revealed hypoalbuminaemia, CRP 7mg/L and ESR 37mm/h. A CT chest revealed an anterior mediastinum soft tissue mass suggestive of necrosis, with multiple ill-defined nodes throughout the lungs.An endoscopy revealed severe gastritis. Oropharyngeal examination revealed pooling of saliva and mucositis. Ceftriaxone was commenced for a presumed infective aetiology.Video fluoroscopy confirmed pharyngeal dysphagia with aspiration. Examination demonstrated a non-fatigable bulbar sounding dysarthria. There was no tongue wasting or fasciculations. Power was globally reduced, with marked proximal upper and lower limb weakness.Nerve conduction studies revealed normal sensation, and most motor nerves had normal conduction velocities with small nerve responses. Electromyography showed areas with denervation.With no improvement in the patient’s condition, anti-tuberculosis and anti-fungal therapy were commenced, with pulsed methylprednisolone for three days followed by 80mg daily to cover for an organizing pneumonia. Subsequent cultures were negative.Progressive weight loss with muscle wasting ensued and later, facial hyperpigmentation was noted in addition to the development of facial, lip and arms swelling. He was rheumatoid factor positive >500 iu/ml, with a raised IgE 2912 g/L and IgG 37.6 g/L. A CT-PET scan revealed intense uptake in the muscles posterior of the neck, tongue and masticators.An MRI scan of his arms revealed several abnormal signals around the shoulder girdle, long muscles of the back, and upper arm, which guided the site for muscle biopsy. This revealed highly abnormal skeletal muscle with frequent atrophic and necrotic fibres overrun by macrophages and T-cell rich inflammation. These findings in addition to serology reporting the presence of anti-SAE antibodies confirmed the diagnosis.Pulsed methylprednisolone, immunoglobulin therapy, and azathioprine were initiated with reducing prednisolone dose.DiscussionThis case of DM with a generalised rash, severe dysphagia and limb subcutaneous oedema were salient features in addition to the presence of anti-SAE antibodies. Anti-SAE has been shown to be present exclusively in DM patients where rash and severe dysphagia are common clinical findings. Our patient presented with severe dysphagia, which can be difficult to manage requiring enteral feeding. Video fluoroscopy was particularly useful in this case helping to stratify the severity of dysphagia and we would urge other clinicians to use this tool when investigating patients with suspected dermatomyositis to avoid potential complications of poor swallow including aspiration pneumonia. The additional imaging modality of PET-CT in our case confirmed the involvement of the muscles of mastication thus could prove a useful tool when investigating involvement of swallowing muscles in patients with anti-SAE DM.Skin features are another common finding in the anti-SAE group and our patient had a heliotrophic rash and shawl sign, which responded poorly to treatment. We describe the additional feature of severe subcutaneous limb and facial oedema, a rare manifestation of the disease, described in only a few other cases. Limb subcutaneous oedema is thought to reflect underlying severe muscle disease, is difficult to treat, and often is unresponsive to conventional treatment. Our case, and several other cases with the presence of limb oedema as reported in a literature search, required treatment with intravenous immunoglobulin and glucocorticoids, in addition with azathioprine and methotrexate.This is the first reported case to our knowledge of a patient with positive ANA, RF, anti-ccp, and anti-small ubiquitin-like modifier activating enzyme (anti-SAE) antibodies.Key learning pointsThe finding of anti-SAE in our case where severe dysphagia was present provides further weight to this antibody being a useful serological marker to identify this subgroup of DM patients. Identifying this antibody may be helpful in creating management strategies for these patients and determining disease progression and prognosis.The presence of limb and facial oedema may be an overlying feature of the anti-SAE group; however, previous cases of limb oedema have not identified this antibody as the SAE test was unavailable. The presence of severe dysphagia and subcutaneous oedema suggests the presence of anti-SAE lends itself to a clinical phenotype of DM that is particularly severe and requires multidisciplinary input.Conflicts of interestThe authors have declared no conflicts of interest.

1559 Chronic Peri-Oral Dermatitis as the First Manifestations of Crohn's Disease

INTRODUCTION: Oral manifestations (OM) have been reported in crohn's disease (CD). The prevalence of these manifestations ranges between 0.5%-50% in adults and 10%-80% in pediatrics. OM of CD may coincide with the intestinal manifestations, or less commonly precede them. Aphthous ulcers, angular cheilitis, swelling of the lips and gingiva, cobblestoning of the mucosa, deep linear ulcers and periodontal disease have been reported in the literature. CASE DESCRIPTION/METHODS: An 18-year-old female with no significant history presented to her PCP with recurrent lip swelling and dryness. She was diagnosed initially with angioedema without urticaria. History of an environmental exposure was inconsistent and testing for hereditary angioedema, including C1 esterase and C4 levels were unremarkable. She was referred to a dermatologist who diagnosed her with peri-oral dermatitis. Treatment with antihistamines, topical steroids, antifungals, antibiotics and petroleum jelly were unsuccessful. She was treated with PO prednisone for a presumed pemphigus vulgaris with symptomatic improvement, but with relapse two weeks after weaning steroids. Due to chronicity of symptoms, a lip biopsy was done and showed a psoriasiform dermatitis with a granulomatous inflammation. Direct immunofluorescent testing was unremarkable. Results were concerning for CD versus cheilitis granulomatosa. She denied GI symptoms, weight loss or family history of IBD. Further testing revealed a normal ferritin, vitamin B12, folate, ANCA, and QuantiFERON-TB. She was referred to GI for further evaluation. An EGD showed a normal esophagus, stomach and examined duodenum. Biopsies from stomach and duodenum were unremarkable. A colonoscopy showed a normal perianal area, colon and terminal ileum. Biopsies from the left colon showed a focal active colitis, foci of neutrophilic cryptitis and focal epithelioid granuloma without dysplasia. Stains for acid fast bacilli and fungi were negative. Biopsies from the right colon and terminal ileum were unremarkable. MRE showed no evidence of active small bowel CD. Given the early onset and widespread distribution, the decision was to start adalimumab. Her OM improved significantly. A repeat colonoscopy with biopsies was unremarkable. DISCUSSION: The recognition of OM can play an important clue in diagnosis and management of CD, especially that an isolated oral disease is uncommon as a first presentation. Infections, nutritional deficiencies and medication side effects are important to consider as differential diagnosis.

Crohn's Disease Presenting as Isolated Lower Lip Swelling

Not available.

SAT-599 Propylthiouracil-Induced Anaphylaxis with Subsequent Tolerance of Methimazole

Background: Although the thionamides have been associated with adverse effects such as cutaneous reactions, hepatotoxicity, and agranulocytosis, anaphylaxis is rarely reported (1). We describe the case of a patient with Graves’ disease, who developed anaphylaxis after treatment with propylthiouracil (PTU), and who subsequently tolerated methimazole. Clinical Case: A 38-year-old woman presented to the emergency department with worsening palpitations, weight loss, and tremor. She was found to be severely thyrotoxic with TSH < 0.01 (normal 0.27-4.20 mU/L), free T4 > 100 (normal 12.0-22.0 pmol/L), free T3 44.2 (normal 3.1-6.9 pmol/L), and TSH receptor antibody 4.0 (normal < 1.8 IU/L). Past medical history was significant for beta-thalassemia trait and two previous early pregnancy losses. Treatment options were reviewed, and initial therapy with an antithyroid medication was recommended. The patient was started on propranolol 20 mg PRN and PTU 100 mg PO TID, chosen because she hoped to become pregnant once euthyroid. She was discharged home with outpatient endocrinology follow-up. After the first dose of PTU, she developed acute onset tongue, lip, and facial swelling, with full-body urticaria. She immediately returned to the emergency department, where she became hypotensive and sustained a syncopal episode. She was treated for anaphylaxis with epinephrine, prednisone, diphenhydramine, and ranitidine. The case was reviewed with specialists from allergy, nuclear medicine, and surgery. In general, it is not recommended to use another antithyroid medication after a severe allergic reaction to the initial agent, as there is a chance of cross-reactivity. Thyroidectomy and radioiodine ablation were deemed risky due to severe thyrotoxicosis and the possibility of precipitating thyroid storm. For this reason, she was challenged with gradually increasing doses of methimazole under close monitoring. She tolerated 1 mg and 5 mg of methimazole while in hospital, and was eventually up-titrated to a total daily dose of 20 mg. There was a resultant reduction of free T4 to 29.9 pmol/L and free T3 to 7.83 pmol/L. Radioiodine ablation was arranged with discontinuation of methimazole one week prior. Thyroid uptake and scan showed 24hr uptake of 87.6% (normal 15-35%) and a diffusely enlarged thyroid gland. She was prescribed 370 megabecquerels of I-131, and was counselled to delay pregnancy for a minimum of six months after therapy and until normalization of TSH. Conclusion: This is the first reported case of anaphylaxis secondary to PTU and subsequent tolerance of methimazole. It highlights challenges that can occur in the management of patients with severe thyrotoxicosis, and the importance of multidisciplinary care in this situation.

Analysis of adverse reactions of antihypertensive drugs registered in the Republic of Crimea for the period 2011-2016

The aim of study was to analyze and investigate the severity of adverse reactions (AR) on drugs used for arterial hypertension treatment in patients living in the Republic of Crimea for the period 2011—2016. Materials and methods . We studied notification cards about AR registered in the regional database of spontaneous messages ARCADe. We analyzed 211 notification cards about AR in patients with proven arterial hypertension diagnosis (ICD code П0—П5). Results. Drugs from the groups of angiotensin-converting enzyme inhibitors and slow calcium channel blockers most often cause AR, among them enalapril and amlodipine are the absolute leaders. AR occurred less frequently in application of drugs from the groups of в-blockers and angiotensin IIreceptor blockers. The study of the clinical manifestations of AR showed that most of them were allergic reactions of varying severity (skin itch, urticaria, swelling of lips, eyelids, tongue). The number of such cases was 70 (33.2 %) from all registered cases of AR on antihypertensive drugs. Examination of the outcomes of AR in patients taking antihypertensive drugs showed that the development of temporary disability was observed in 19 (9 %) cases, the hospitalization of outpatients as a result of AR development on drugs was required in 7 (3.3 %) cases, and in 2 cases patients had to prolong hospitalization. Life-threatening conditions in the form of Quincke’s edema were reported in 13patients. In the remaining 170 (80.6 %) cases AR were not serious and did not cause the above consequences. Assessment of AR severity revealed that mild AR were most common, much less often antihypertensive drugs caused severe AR (the incidence ranged from 5 to 13 %, depending on the method for severity assessment). Conclusion . The obtained results confirm our previously published data that in the structure of AR on antihypertensive drugs, bulk of the reports associated with angiotensin-converting enzyme inhibitors. Most often antihypertensive drugs caused mild AR.

Orofacial Granulomatosis in an Orthodontic Patient: a Case Report

A case is presented of a 14-year-old male patient being treated with a fixed orthodontic appliance who presented with a persistent upper lip swelling. The patient was experiencing discomfort from the appliance and the appliance was debonded. A diagnosis of orofacial granulomatosis was established and the patient was managed carefully with a cinnamon- and benzoate-free diet leading to resolution of the swelling. Orofacial granulomatosis is a rare condition and its presentation, diagnosis, and management are discussed. CPD/Clinical Relevance: Orthodontists should be aware of the presentation of orofacial granulomatosis and the possible challenges associated with managing these patients.

Melkersson Rosenthal Syndrome: a case report and review of the literature

Melkersson Rosenthal Syndrome (MRS) is a rare neurological disorder characterized by swelling of the face, particularly one or both lips (granulomatous cheilitis), facial muscle weakness (palsy) and a fissured tongue. We present a patient with Melkersson Rosenthal Syndrome, highlighting the clinical triad of symptoms and management. A74-year-old Nigerian male presented to the Oral Medicine Clinic of the University of Benin Teaching Hospital for evaluation of right-sided facial numbness, inability to close the right eye, left facial deviation suggestive of a lower motor neuron type facial palsy of 4 days, and painless swelling of the upper and lower lips for seven days. On examination, he was found to have swelling of the upper and lower lips, multiple fissures on the tongue, right facial paresthesia, and an isolated right-sided facial nerve paralysis. He was empirically managed with 50mg prednisone daily for seven days, which was tapered over two weeks and neurobion 1 tablet daily for a month. This resulted in remission of lip swelling, however fissured tongue remained. Two months later, facial deviation had become less apparent. Patient is being followed up in the outpatient clinic. Melkersson Rosenthal Syndrome is a rare disorder with features of facial swelling, facial nerve palsy and fissured tongue. Some affected individuals may have all three of these features and others may have only one or two. The diagnosis of MRS can be made clinically when there is a complete triad of symptoms as reported in our patient.Keywords: Melkersson Rosenthal Syndrome, clinical symptoms, management

A rare case report on telmisartan-induced angioedema and mouth ulcers

A 67-year-old hypertensive female was hospitalized for painful, bleeding mouth ulcers, angioedema (lip swelling and glossitis), and dysphagia. A history reported by the patient and recent medication history revealed the onset of the symptoms on the initiation of telmisartan for hypertension. Telmisartan was discontinued, and the patient was managed symptomatically for pain, mouth ulcers, glossitis, and dysphagia. The symptoms completely resolved over 10 days of drug withdrawal. As per the World Health Organization and Naranjo adverse drug reaction assessment, telmisartan had a “Probable” association with the oral toxicity observed in this patient.

A Case of Glandular Cheilitis Presenting with Recurrent Swelling of the Lips during the Course of Rheumatoid Arthritis

Cheilitis glandularis (CG) is a rare disorder of unknown etiology charactarized by inflamation of the minor salivary glands of the lower lip. Clinically, three variants have been described: cheilitis glandularis simplex, cheilitis glandularis suppurativa, and cheilitis glandularis apostematosa. Most cases are diagnosed as the simple form of glandular cheilitis, which is characterized by enlarged excretory ducts, induration, and enlargement of the salivary glands as well as production of a rather mucopurulent saliva. The diagnosis of CG is done with clinical and pathologic correlation. A swollen lip is common in clinical practice, but the differential diagnosis and management of the condition may be challenging. Lip swelling may be related to a local or systemic condition. We hereby report an unusual case of CG of lips in a female patient visited the deparment of Internal Medicine, Ege Univercity. She was being followed up with the diagnosis of rheumatoid arthritis and presented with recurrent swelling of the lips repeatedly. She had previously been followed up for angioedema because of recurrent swelling of the lips. The patient's laboratory tests were normal and lip biopsy was consistent with glandular cheilitis. The aim of this article is to discuss the differential diagnosis of glandular cheilitis.

Case report on Fluconazole Induced stevens -Johnson Syndrome

Stevens-Johnson syndrome (SJS) is a rare cutaneous reaction characterised by mucosal erosions and epidermal detachment of about less than 10% of the total area of body skin. The incidence of SJS is approximately 1–6 cases per million person-years. The incidence of fluconazole induced SJS is less than 1%. Here the patient came with complaints of scaly lesions over chest and axilla following which he was started on topical antifungals (Lulliconazole) and antihistamines with the suspicion of Tinea versicolor. Though few lesions resolved on treatment, the patient later developed a small scaly macule over the left upper arm. Thus the patient upon further evaluation was given a stat dose of Tablet Fluconazole 400mg. Following this, the patient developed swelling of lower lips and tip of the glans and erythema over palms. This was later confirmed as Stevens-Johnson syndrome. The reaction was suspected to have occurred in association with Fluconazole. Fluconazole had a probable causal relationship with Stevens Johnson Syndrome as per ALDEN Algorithm in the EuroSCAR study. As per the Naranjo ADR probability scale, the causality assessment score for this reaction was found to be 5 which indicates a probable causal relationship. Clinicians must be more vigilant while prescribing Fluconazole by conducting regular monitoring of such patients and carrying out early diagnosis of such reactions.

Characteristics of the salivary microbiota in cheilitis granulomatosa.

Background Cheilitis granulomatosa (CG) is a disturbing and persistent idiopathic lip swelling. The cause and treatment has not been wholly elucidated. Some reports infer that CG is mainly associated with dental infection but no firm or reliable microbiological evidence has been provided for a causative organism. This study aimed to evaluate whether microorganisms contribute to the etiology of CG in order to inform appropriate treatment options in clinic. Material and MethodsUnstimulated saliva was collected from 15 CG patients who were diagnosed clinically and pathologically and 15 healthy controls (HC). DNA was extracted from the precipitate of the centrifuged saliva for 16s rRNA high-throughput sequencing using the Miseq PE300 platform. The distribution of the microbiome between the two groups was compared. ResultsCG patients had a greater microbial flora that was more diverse than the HC. Prevotella, Alloprevotella, Porphyromonas, Actinomyces, Rothia, Fusobacterium, Haemophilus, and Aggregatibacter had a significantly higher abundance in CG patients. In contrast, Streptococcus and Campylobacter were the most abundant genera in HC with a mean relative abundance of 63% and 2%, respectively. The microbiological network indicated that most of the bacteria that were enriched at greater levels in CG patients were likely to be Prevotella, Actinomyces, and Rothia. These have been shown to co-exist with other bacteria. Conclusions: The composition and structure of bacterial communities in CG patients were different from HC. Most of the genera observed in CG patients were associated with periodontitis and pulp infection. These findings might be helpful in understanding the etiology of CG. Further study will be needed to confirm these findings and explore the underlying pathological mechanism. Key words:Cheilitis granulomatosa, 16s rRNA high-throughput sequencing, salivary microbiota.

Perindopril-induced angioedema of the lips and tongue: a case report

BackgroundAce inhibitor-induced angioedema, characterized by sudden-onset swelling of the mucous membrane, skin, or both, is a rare occurrence in the Kingdom of Saudi Arabia. Because of its safety and efficacy, perindopril is a commonly prescribed angiotensin-converting enzyme inhibitor. Here we describe the clinical manifestations, management, and outcome of perindopril-induced angioedema of the lips and tongue in a 65-year-old Saudi man.Case presentationA 65-year-old Saudi Arab man presented to an emergency department with lip and tongue swelling and dysphagia. There were no systemic symptoms and no past history of a similar event. He had been consuming perindopril 5 mg and amlodipine 5 mg for the last 3 weeks: brand name, Coveram, from the company Servier (Ireland) Industries Ltd.; one tablet of Coveram contains 3.395 mg perindopril corresponding to 5 mg perindopril arginine and 6.935 mg amlodipine besilate corresponding to 5 mg amlodipine. A physical examination revealed considerable swelling of his lips and tongue.Examinations of other systems, including his cardiovascular and respiratory systems, revealed unremarkable findings. All laboratory parameters were also normal. Electrocardiography demonstrated sinus rhythm, a normal P axis, and V-rate of 50–99.A clinical diagnosis of perindopril-induced angioedema was made, and perindopril was discontinued. The angioedema resolved completely after the administration of antihistamines and corticosteroids.ConclusionsAngioedema caused by angiotensin-converting enzyme inhibitors is an uncommon occurrence in Saudi Arabia. The findings from this case are expected to raise awareness about this condition among clinicians in this country.

Lip Leishmaniasis with Oral-mucosal Involvement: A Case Report

Statement of the problem: Nowadays leishmaniasis is a common infectious disease around the world which is caused by a protozoan parasite named Leishmania. This parasite is transmitted to human by an infected female sand fly. This disease has three clinical forms; mucosal forms are rare in Iran. Purpose: The purpose of this study was to report a case of leishmaniasis with lip and oral mucosal involvement that healed successfully. Results: This article is about patients with lip leishmaniasis with oral-mucosal involvement, clinical features were diffuse lip swelling and ulcer in right lower lip vermilion and buccal mucosa. A smear was caught which proved the diagnosis of leishmaniasis. Thereafter, the patients were referred to a dermatologist for treatment and follow up showed complete healing of the lesions. Conclusion: Oral mucosal leishmaniasis is rare. However, the swelling may cause different complications like loss of teeth or respiratory obstruction. It should be noted that time is a key point in treatment. So, it is necessary for us to train the differential diagnosis of chronic ulcerated oral lesions to our dentists.

PERSISTENT LIP AND FACIAL SWELLING IN A 9 YEAR OLD BOY

Introduction Orofacial Granulomatosis (OFG) is a chronic granulomatous condition characterized by persistent or recurring swelling of soft tissues in the face. It is thought to be caused by a delayed hypersensitivity reaction involving a Th1-mediated immune response. There have been reports of cases associated with Sarcoidosis and Crohn's disease. Case Description A 9-year old male presented to clinic with 15 months of lip and facial swelling. Episodes last 2-3 weeks with resolution periods of 1-2 weeks. Antihistamines failed to improve the swelling. He has normal growth and development. He denies any fevers, headaches, eye redness/pain, oral/nasal ulcerations, difficulty swallowing, decrease in physical conditioning, nausea/vomiting/diarrhea, or bone/joint/muscle complaints. He was started on high dose steroids which improved his lip swelling but as the prednisone was tapered, the swelling returned. He had a mild elevation in Ig G4 and CRP, positive histone antibodies, and elevated C3/C4 and Ig G levels. His Hereditary Angioedema, Sarcoidosis, and Crohn's disease evaluation were negative. Eventually a biopsy of the vestibule of the anterior mandible was done which showed non-necrotizing granulomas. Discussion OFG symptoms can be indistinguishable to those caused by angioedema, contact dermatitis, melkersson-rosenthal syndrome, and lymphangioedema. OFG is made from a diagnosis of exclusion as biopsy cannot distinguish between OFG and Crohn's or Sarcoidosis. Treatment of this condition can be challenging and include steroids, NSAIDs, antibiotics, and TNF-alfa inhibitors. The goal of this case is to provide insight on this uncommon condition whose symptoms present as known sequela of frequent diseases that allergists encounter in their practice.

NOT ALL LIP SWELLING IS ANGIOEDEMA

Introduction Cheilitis granulomatosa (CG) is a rare form of recurrent or persistent swelling of one or both lips and one form of orofacial granulomatosis. It is important for allergists to consider the need for mucosal biopsy in patients initially referred for angioedema, especially if the lip swelling has been persistent. Case Description A 35-year-old female presented with 2 months of constant lip swelling. Swelling was worse in the morning with some improvement in the evening although was always present. Early in the course, she was treated with prednisone for one week and antihistamines with initial improvement, but the swelling recurred. Lip swelling was not associated with any tongue swelling, hives, difficulty breathing, or facial paralysis. She had no previous history of angioedema, allergic rhinitis, or asthma. On physical exam, right lower lip was enlarged without any oropharyngeal or tongue swelling. There was mild desquamation of the lips. Patient was initially treated with topical steroids given suspicion for contact dermatitis related to frequent lip balm use. Given persistent symptoms, a lip biopsy was recommended, which was consistent with CG. The patient is now receiving intralesional triamcinolone with improvement of the swelling. Discussion The differential diagnosis for lip swelling is vast. CG along with other granulomatous diseases should be considered when the swelling is persistent with or without associated surface epithelial changes, both of which should not be seen in angioedema of any cause. It is important not to miss this diagnosis due to its association with Crohn's disease.

ANAPHYLAXIS TO COFFEE FROM A SPECIFIC GEOGRAPHIC REGION

Introduction We present anaphylaxis to coffee from one region with tolerance to coffee from elsewhere. This is relevant, as foods from various geographic regions are widely available. Case Description 28-year old Saudi Arabian male presented with throat closing, chest tightness, wheezing with airborne exposure to Arabic coffee. When drinking Arabic coffee, he has immediate lip swelling. Denies hives, pruritis, breathing issues. Drinks black Starbucks coffee (House blend, Latin America) without symptoms. Arabic coffee (Yemen) is brewed with cardamom, ginger, milk. Patient tolerates cardamom, ginger, dairy. He has nasal allergies triggered by cats, trees (skin testing, 2016). Significant physical exam findings: dark circles, Dennie's folds, moderate nasal turbinate edema. Skin testing Positive to bell pepper (7/10), walnut (7/10), hazelnut (7/20), brewed Arabic coffee containing cardamom, ginger, milk (13/30). Negative to brewed Starbucks coffee (0/0); cow's milk (0/0). Histamine 7/20 Saline 0/0 Discussion This patient has history consistent with anaphylaxis to Arabic coffee, but not to Latin American coffee. He tolerates the components of Arabic coffee (cardamom, ginger, milk) in other foods. Skin test was positive to brewed Arabic coffee and negative to Latin American coffee. We recommended avoidance of Arabic coffee, self-injectable epinephrine, Albuterol inhaler. To our knowledge, this is the first description of a patient being sensitized to coffee from one region but not another, perhaps due to inherent differences in coffee from disparate regions or to variations in processing. This patient also has tree nut allergy. We were unable to determine whether the Arabic coffee was cross contaminated with any tree nuts.