Recurrent Swelling Research Articles

Endoscopy-Assisted Surgery for Mandibular Keratocystic Odontogenic Tumor Presenting as Recurrent Hemifacial Swelling.

Endoscopy-Assisted Surgery for Mandibular Keratocystic Odontogenic Tumor Presenting as Recurrent Hemifacial Swelling.

39 Pediatric monoarthritis of the elbow: atypical onset of a common disease

Abstract Background Oligoarticular type of juvenile idiopathic arthritis (oligoJIA) is considered the most common type of JIA. It usually affects one or < 4 joints. The knee is the most common affected joint followed by the ankle and wrist. Oligoarticular JIA presenting with erosive elbow monoarthritis as an initial sign of the disease is a rare condition which needs prompt diagnosis and management. Objective To recognize that isolated chronic elbow monoarthrits is an atypical presentation for oligoJIA. Case presentation: A 14-year-old volleyball player female child presented with chronic recurrent left elbow swelling of six months’ duration. No history of morning stiffness, constitutional symptoms, other joint swelling, uveitis, or skin manifestations were reported. On examination there was mild cubitus valgus with elbow effusion associated with limited last degree elbow extension with no other signs of inflammation or infection (redness, tenderness, hotness). Hypermobility was noted in both elbows and knees. Patient was advised to rest the elbow and symptomatic treatment was recommended. On follow up there was mild improvement of the swelling, so further investigations were ordered. Erythrocyte Sedimentation Rate (ESR), C-reactive protein (CRP), Rheumatoid factor (RF) and antinuclear antibody (ANA) showed average normal values. X-ray of the elbow showed degenerative changes. Musculoskeletal ultrasound showed mild elbow effusion and magnetic resonance imaging (MRI) was ordered revealing effusion and early joint erosion at the ulnar articular surface with no synovial hypertrophy. Arthroscopy was recommended to detect the underlying pathology and histopathology revealed chronic active synovitis suggesting JIA. Non-steroidal anti-inflammatory drugs (NSAIDs) were recommended but one month later knee arthritis was noted, so methotrexate was started and the condition started to improve. Results We report a 14-year-old female child who presented with atypical isolated chronic swelling of the elbow and was diagnosed by arthroscopy and histopathology. Non-steroidal anti-inflammatory drugs (NSAIDS) were tried as a first line treatment followed by methotrexate. Conclusion Chronic elbow monoarthritis is an atypical onset of oligoJIA and its diagnosis is important to prevent irreversible joint damage. Synovial biopsy can have a major role in the diagnosis of atypical monoarticular JIA and can help in early diagnosis and management improving the outcome. The implication to policy, practice, research, and advocacy: Monoarthritis of the elbow as initial sign of oligoJIA is an atypical onset of this disease and in the absence of systemic signs or other clinical indicators determining the exact cause of monoarthritis in children might be difficult. Synovial biopsy can have an important role in the diagnosis of monarticular arthritis in pediatric patients.

A Rare Cause of Knee Pain and Swelling: Venous Malformation Masquerading as Diffuse Pigmented Villonodular Synovitis

This case report recounts the history of a 21-year-old patient who presented with a 19-year history of recurrent unilateral knee pain, swelling, and restricted movement. At age 10, the patient had a diagnostic knee arthroscopy which resulted in a provisional diagnosis of pigmented villonodular synovitis. The patient was treated for this condition for several years through medical and surgical means. Despite treatment, symptoms did not resolve. Only after the case was discussed by the multidisciplinary team was further imaging through ultrasound (US) suggested. A new diagnosis of venous malformation (VM) was made after US, and the patient was treated accordingly. This case report adds to the literature examining intra-articular VMs, the use of multimodal imaging in the diagnosis of intra-articular lesions of the knee, and the importance of multidisciplinary discussion in the diagnosis of complex knee pathology.

P42 Primary Sjogren’s Syndrome (pSS) in a seronegative adolescent female with family history of BehÇet’s disease: A challenging disease to diagnose Case-based discussion

Introduction/BackgroundSjogren’s Syndrome (SS) is heterogenous chronic autoimmune disease with symptoms ranging from organ-specific to variable non-specific systemic features. It is a rare disease in childhood and can be very difficult to diagnose, especially in seronegative patients, with the absence of any specific diagnostic investigation or criteria. We are presenting a seronegative juvenile Primary Sjogren’s syndrome (pSS) patient with all the challenges we had to confirm the diagnosis and a highlight on the role of MDT as the most sensitive tool.Description/MethodA 14-year-old female patient was first referred to our Paediatric and adolescent rheumatology department at the age of 10 with history of recurrent bilateral painful parotid swellings since the age of 3 with absence of ANA, RF, Anti-SSA-Ro and Anti-SSB-La antibodies repeatedly. From 2016 till 2019 she was under the care of ENT with recurrent complicated bilateral ear infections. Episodes tend to occur every 2-3 weeks and last between a few days to 2 weeks. Her events were associated with fever, fatigue, shivering, joint and leg pain, cervical lymphadenopathy, infrequent folliculitis, and headaches. No history to indicate mouth or eye dryness or significant repeated ulcers. Both of our patient’s mother and maternal grandmother have Behçet’s disease. Further blood investigation only showed mild hypergammaglobulinemia with mildly elevated ESR and CRP, normal LFT, U&Es, complements, muscle enzymes and thyroid function. HIV serology was negative and serum IgG4 subclass was normal. Many USSs confirmed parotitis. Parotid gland biopsy was not specific and indicated chronic parotitis with diffuse infiltration of mature lymphocytes, periductal lymphocytic inflammation and exocytosis of lymphocytes into the ductal epithelium. Focus score nearly reaching 50 cells. Plasma cell staining ruled out IgG4 related disease. Ophthalmic assessment ruled out eye involvement by the appearance of her eyes. The Oral Medicine Team confirmed the appearance of dry mucosa with minimal salivary flow from the parotid ducts, though it has not been subjectively reported to cause any problem before. Failed trial of colchicine, never been off-prednisolone for at least the last 2 years, tried Methotrexate which resulted in significant but not full improvement. Now she is on azathioprine and Hydroxychloquine, but still reporting flares. Escalation in treatment is required though there are some concerns about previous medication compliance, the actual severity of symptoms reported and the lack of current suitable accommodation.Discussion/ResultsDiagnosing pSS is quite challenging especially in seronegative patients due to the possibility of other non-autoimmune diseases causing recurrent parotid gland swellings, especially Recurrent Juvenile Idiopathic Parotitis with very young age of onset. With the persistence of symptoms and lack of an underlying infection, our patient ended up having a parotid biopsy. This was generally not specific, with a focus score close to the 50 mononuclear cells required as evidence of exocrine inflammation. This further added to the challenge of confirming an underlying autoimmune disease. Nine years into the disease course, our patient was confirmed to have objective evidence of dry mucosa and decreased salivary flow from the parotid ducts. This shows how important it is to assess salivary and lacrimal gland dysfunction in a child with suspected pSS, despite the absence of typical xerophthalmia and xerostomia. Considering the non-typical features (sero-negativity and the non-specific histopathology findings), our patient clinical picture was discussed in an MDT meeting including ENT, Histopathology and Rheumatology and the diagnoses of pSS was confirmed. The chronicity of the disease, significant improvement of the flare frequency and severity while on immunosuppressants, the reoccurence of significant flares when the patient was off-treatment (personal decision), and the objective evidence of poor salivary flow all supported the diagnosis of pSS later in the disease course. This case shows us how the typical adult features of pSS should not be strictly applied to the juvenile form of the disease. It also highlights the importance of having an MDT as the most sensitive available tool to confirm the diagnosis.Regarding lack of subjective full response, how do you think we can prove this objectively and what would be the most appropriate next step in treatment escalation?Key learning points/ConclusionA high index of suspicion is crucial to reach the diagnosis and to avoid irreversible damage in children with SS.Quadrable sero-negativity should not be looked at as an absolute exclusion criterion of juvenile pSS.Exclusion of other more common and diverse causes of parotitis is crucial.The typical adult features of SS should not be strictly applied to the juvenile form of the disease.MDT approach is the most sensitive tool to diagnose SS.When there is no subjectively reported ophthalmic or oral dryness, we would advocate sending suspected children of pSS for formal assessment of it- we understand the age-related challenges.Considering its rarity, we need to improve the general awareness of the juvenile form of the disease among the population and the medical practitioners.

MR-lymphangiography identifies lymphatic pathologies in patients with idiopathic recurrent cervical swelling.

BackgroundIdiopathic recurrent cervical swelling may be caused by lymphatic abnormalities.MethodsTen patients (9 females, mean age 51.2 ± 7) with idiopathic recurrent cervical swelling underwent MR‐lymphangiography (MRL). MR‐lymphangiograms were evaluated regarding lymphatic anatomy and flow. Individualized treatment was recommended according to MRL‐findings.Results8/10 patients presented with left‐sided, 2/10 with right‐sided swelling. Pathological lymph‐flow was identified in all cases: thoracic duct dilatation in patients with left‐sided and right lymphatic duct dilatation in right‐sided swelling, accessory thoracic lymphatics in 7/10 and reflux in 8/10 cases. In two cases, a lymphatic thrombus was identified.After treatment, symptoms resolved completely in 6/10 cases and partially in 1/10 cases. The remaining three patients have intermittent swellings but have no treatment wish.ConclusionIdiopathic recurrent cervical swelling can be caused by lymphatic anomalies. MRL displays impaired lymphatic drainage, lymphatic vessel dilatation, and chylolymphatic reflux as hallmarks of this condition and may aid in targeted treatment planning.

Recurrent Submandibular Gland Swelling Relieved by Antihistamine Medication: Is It Eosinophilic Sialodochitis?

Sialodochitis fibrinosa is a disease characterized by salivary duct obstruction. A 21-year-old male presented with a painful submandibular gland (SMG) swelling. Serum eosinophilia and Whartons’ duct dilatation with strong enhancement were observed on contrast CT. Core needle biopsy (CNB) for SMG parenchyma revealed lymphocytic infiltration between dilated intralobular ducts. In another case, a 39-year-old male complained of recurrent major salivary glands swelling for ten years with an itching sensation on the overlying skin of the salivary gland. Enhancement of both SMG parenchyma, dilatation of both Wharton’s ducts and elevated serum eosinophilia were observed on contrast CT study. CNB for SMG parenchyma revealed lymphocytic infiltration with many eosinophils around a markedly dilated interlobular duct. The recurrent SMG swelling in both cases were relieved by antihistamine medication, warranting suspicion that these cases might organ-specific eosinophilic disease. We dicuss these two cases with a literature review.

A RARE CASE OF MANDIBULAR ASPERGILLUS OSTEOMYELITIS IN AN IMMUNOCOMPETENT PATIENT

A 57-year-old White woman was referred for evaluation of a painful recurrent swelling with purulent drainage in the anterior mandibular alveolar ridge. The patient reported that surgical interventions and antibiotic therapy were performed previously in other services without success. Otherwise, the patient was systemically healthy. A new surgical debridement was performed, and histologic examination showed osteomyelitis associated with <i>Aspergillus</i> species. The patient was referred to an infectious disease physician and treated with oral itraconazole (400 mg per day) for 3 months. Complete resolution was achieved, and the patient was followed for 15 years and no recurrence was observed. The patient was rehabilitated with dental implants and overdenture prosthesis. Other microorganisms, such as <i>Aspergillus</i>, should be considered in cases of mandibular osteomyelitis that do not heal after surgical debridement and antibiotic therapy.

A Case Report of Management of Multiple Peri-orbital Cysts (Eccrine Hidrocystoma) in a 35-Year Old Woman

Background: Hidrocystoma is a rare benign swelling(tumour) of sweat glands also known as the cystic form of hidroadenoma, cystadenomas, sudoriferous cysts, and Moll’s gland. It has no racial or sex predilection. Its rarity and site of occurrence makes its management challenging due to limitation in knowledge of making diagnosis, treatment, availability of drugs and interventions and aesthetic concerns.&#x0D; Presentation of Case: A 35 year old Nigerian woman from the Igbo ethnic group with tertiary level of education who presented to the dermatology outpatient clinic with a 10 year history of recurrent peri-orbital swelling associated with occasional pruritus. There was no history of similar lesion in any family member. Patient had resorted to self-medication and consulted different doctors prior to being referred to the dermatologist. Her physical examination was normal. She was also counselled about the disease and management options. She subsequently received seven sessions of the chemo-ablation using trichloroacetic acid (TCA) with remarkable improvement and has had no recurrence of lesions for the past 1 year.&#x0D; Conclusion: Hidrocystomas can be successfully treated in our environment.

Talus osteomyelitis by Candida krusei with multiple huge cystic lesions: a case report and review of literatures

BackgroundOsteomyelitis due to Candida krusei are extremely rare, given that only six cases have been reported, all of which are limited to the patients with immunocompromising risk factors. Here we report a case of C. krusei osteomyelitis in an immunocompetent patient, presenting with multiple huge cystic lesions of talus.Case presentationA 66-year-old female presented with one year history of painful swelling of right ankle and a draining sinus around lateral malleolus. Five months and three months ago, she had undergone arthroscopic synovectomy and bursectomy which revealed no causative organism. Open bursectomy with sinus tract excision was performed and intravenous antibiotic was administered. Two year after the surgery, the patient revisited the clinic for recurrent painful swelling with pus drainage at the same location. Multiple huge cystic lesions with osteolysis and sclerotic rim of talus were found and C. krusei was isolated from tissue culture. The patient received surgical debridement and prolonged antifungal treatment comprising caspofungin and voriconazole.ConclusionsIn this case, C. krusei infection showed atypically aggressive osteolysis shown as multiple huge cystic abscess. High index of suspicion is critical for early diagnosis and treatment to prevent such devastating results even in an immunocompetent patient.

Clinico surgical experience of thyroglossal duct cysts in a tertiary care hospital of Southern Odisha

Introduction: The thyroglossal duct cyst (TDC, also known as the thyroglossal tract remnant, or TTR) is a well-known developmental anomaly that affects roughly 7% of the population. It often manifests as a movable, painless lump. Objectives: to report our 2 years of clinical experience with thyroglossal cysts in terms of clinical aspects and surgical findings. Materials and methods: This study was conducted in Department of General Surgery, MKCG Medical College and Hospital, Berhampur over a period of 24 months from July 2019- June 2021. Results: The majority (72.72%) were under the age of fifteen. Patients aged 16 -30 years made about 24.24% of the patient population. Males made up 63.63 % of the population, while females made up 36.36%. 88% of patients had neck swelling when they arrived. 15.15% of patients exhibited erythema/redness over swelling, and 18.18% felt tenderness. 15.15% of patients at presentation had painful swelling and deglutination. 18.8% of patients reported having experienced recurrent neck swelling in the previous two years, while 12.12 % only had recurring discharge from the midline neck.

Melkersson-Rosenthal Syndrome: A Mysterious Path to Recurrent Facial Palsy

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous syndrome of unknown etiology with chronic and progressive course, and clinical diagnosis is usually by exclusion. Rarely, it presents with the full triad of recurrent or persistent facial swelling, relapsing facial palsy, and a fissured, geographic tongue. It is rarely possible to observe all aspects of the classical triad at the same time, since these symptoms may appear in different times of life cycle. Although etiology of MRS is unclear, various factors such as infections, genetic predisposition, immune deficiency, food intolerance and stress have been held responsible. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. The therapeutic options are varied, though most commonly include corticosteroid therapy and nerve decompression surgery

Steroid-eluting Stent Placement for Refractory Stenosis of Parotid Megaduct After Sialodochoplasty.

Salivary megaduct with stricture is characterized by recurrent pain, swelling, and infection, leading to reduced quality of life. Surgical management includes sialodochoplasty, however, recurrence is common and repeated surgery can lead to further scarring. In the sinuses, drug-eluting stents (DES) are used to reduce scarring after surgery, yet no such technology exists for salivary megaduct with stricture.We trialed DES for this condition with promising results which may pave the way for future development.

De-differentiated giant thigh liposarcoma disguised as recurrent lipoma; a case report

Introduction and importanceLipomas are one of the commonest soft tissue tumors which usually arise from subcutaneous plane. Lipomas can progress to one of the three types of liposarcomas. Dedifferentiated liposarcomas have metastasis potential, risk of recurrence and increased risk of mortality. The culture of taking every lipoma as benign should be seriously considered with different approaches as it can lead to sarcomatous degeneration in some cases.Case presentationA 78 years old male patient present to our hospital with recurrent left thigh swelling of two years duration with limping and sense of heaviness on left side. He has history of previous surgery for the same complaint on the same site two times, six and four years ago. Histological diagnosis in both cases was lipoma. Objectively he has 20 × 60 cm non tender, soft to firm, slightly mobile swelling on left thigh spanning anterior, lateral and posterior compartment but no inguinal swelling. Fine needle aspiration cytology from the mass reported sarcoma. CT-scan stated diagnosis as sarcomatous tumor.Clinical discussionLipoma is commonest soft tissue tumor arising from adipocytes more often in young adults, women and overweight population. Well-differentiated liposarcoma can progress to dedifferentiated liposarcoma over time which is slightly aggressive than its precursor. Management of liposarcoma depends on stage, site and histology. Surgical resection with radiotherapy is the standard of care for localized dedifferentiated liposarcomas.ConclusionIt is difficult to differentiate between giant lipoma and well-differentiated liposarcoma with physical examination and imaging. Histopathologic examination in uncertain conditions will decrease morbidity and mortality from the more aggressive forms of liposarcomas.

A large submandibular salivary stone: A case report

Sialolithiasis accounts for the most cases of salivary gland obstruction which leads to recurrent painful swelling of the involved gland which often exacerbates while eating. Stones may be encountered in any of the salivary glands but most frequently in the submandibular gland and its duct. This is a case report of a 40-year-old male patient who had a submandibular sialolith. The sialolith was removed with intraoral approach and no post-operative complications were noted. The article also reviews the various available diagnostic modalities and treatment options.

Alveolar soft part sarcoma–presenting as an unusual scapular swelling- A rare case report

Alveolar soft part sarcoma (ASPS) is a very rare soft tissue tumor of unknown histogenesis and has propensity for recurrence and distant metastasis. It commonly occurs in the head neck region and on extremities in adolescents and young adults. Less number of cases have been reported in the scapular region so far. Till now, no definite treatment regime is available for ASPS; early diagnosis plays a pivotal role in its management and patient outcome. Here, we are presenting a case of 23 years old male presenting with a recurrent swelling in right scapular region. It was diagnosed as ASPS on the basis of radiology, histomorphology and immunohistochemistry.

Abstract #1183932: Management Challenge in Hyperphosphatemic Tumoral Calcinosis

Tumoral calcinosis (TC) is a rare metabolic disorder causing abnormal calcium salt deposition in different peri-articular soft tissue regions. The most common site is lateral hips. TC can be primary normo-phosphatemic or primary hyperphosphatemic. It can be secondary also, seen commonly in chronic renal failure. We describe a case report of 14 years old girl who presented with primary hyperphosphatemic tumoral calcinosis with recurrent swellings even after surgery and medical therapy. A 14-year-old girl presented to us with recurrent soft tissue mass and persistent hyperphosphtemia associated with pain and decreased movements. She was operated twice for this soft tissue. Her medical record stated that patient had undergone 6 surgeries till date for these periarticular swellings at multiple sites (right elbow, left hip, right hip and left knee) but it recurs after 3-4 months of surgery and progressively enlarges in size. Physical examination revealed firm tender 9 X 4 cm oval shaped swelling in the left greater trochanteric area with restriction in abduction, internal rotation and rotator movements at hip joint and 4 X 4 cm circular hard, non-sessile mass over medial aspect of left knee joint with slight difficulty in complete flexion of knee. Biochemical work up showed hyperphosphatemia with normal serum calcium and low iPTH levels, low serum 25 hydroxyvitamin-D and slightly high 1,25 vitamin D with calculated TmP/GfR of 5.7 mg/dl. C-terminal FGF23 level was grossly elevated to 1200 RU/ml. Histological evaluation of previously surgically excised soft tissue mass revealed fibrocollagenous stroma with clusters of crystalline and amorphous calcium enclosed by band of histiocytes and lymphocytes compatible with findings seen in tumoral calcinosis. Presently the patient is maintaining the near normal phosphate level with oral phosphate restricted diet and multiple phosphate binder and acetazolamide. Hyperphosphatemic Tumoral calcinosis is a disorder of dysregulated phosphate metabolism characterized by hyperphosphatemia and deposition of calcium salts in soft tissue regions of the body usually in the periarticular sites. FGF23 is a 251-amino acid peptide secreted by osteoblasts, osteocytes and erythroid precursor cells of the bone marrow that plays a critical role in phosphate regulation. In the proximal tubule of the kidney, FGF23 binds to the FGF receptor 1 (FGFR1) and its co-receptor KLOTHO, downregulating expression of the sodium-phosphate cotransporters NPT2a and NPT2c, which leads to phosphaturia. Additionally, FGF23 inhibits 1-alpha-hydroxylase and stimulates 25-vitamin D-24 hydroxylase, resulting in decreased 1,25-(OH)2-vitamin D (1,25D), the active form of vitamin D. Recently an entitity named autoimmune hyperphosphataemic tumoral calcinosis has been reported in which pathogenetic autoantibodies mediate FGF23 resistance.Treatment is to lower serum phosphorus by blocking dietary phosphate absorption and increasing renal phosphate excretion. Surgery is usually reserved for patients with significant pain or restricted movements of joint.

Superficial parotidectomy: three-year experience using modified Blair's incision

Background: This study presents a series of parotidectomy for superficial benign parotid tumors.Methods: 18 patients had freely mobile superficial benign parotid lesions of grade zero on House-Brackmann facial nerve grading system underwent parotidectomy through modified Blair incision, with meticulous dissection of the main trunk and branches of facial and the greater auricular nerves with parotid duct sparing. Postoperative (PO) facial nerve functions were assessed and graded after patient was fully conscious, on the 5th PO day, and 6th PO month. Collected data included operative time, intraoperative blood loss, duration of hospital stay, wound drainage and PO complications.Results: Mean operative time was 115 (±20.4) minutes and mean intraoperative blood loss was 250 (±60). PO analgesia was provided as non-steroidal anti-inflammatory drugs injection on patient's request. Mean duration of PO hospital stay was 8 (±1.9) hours and of wound drainage was 3 (±0.7) days. No wound infection nor seroma had developed and stitches were allowed for spontaneous removal. Immediate postoperatively 4 patients (22.2%) had incomplete facial nerve weakness; 3 of grade II and one of grade III, at the 5th day PO, two patients still had grade II nerve affection and at the 6th PO month, one patient (5.5%) still had grade II nerve affection.Conclusions: superficial parotidectomy through modified Blair's incision is feasible, safe and allows nerve, vessels and duct sparing even for cases with recurrent swelling. Incomplete facial weakness was encountered in only one patient at the 6th month PO, but was of grade II.

Chronic Osteomyelitis of the Jaw: Pivotal Role of Microbiological Investigation and Multidisciplinary Management—A Case Report

A 15-year-old girl with a history of recurrent painful orofacial swelling was diagnosed on the basis of clinical findings, histopathological examination and imaging modalities as having primary chronic osteomyelitis of the jaw. Initial microbiological samples were performed but were inconclusive. She received multiple empirical antibiotic therapies and NSAIDs for 3 years without complete remission. Only MALDI-TOF (Matrix-Assisted Laser Desorption/Ionization–Time Of Flight) analysis after additional multiple microbiological bone samples with adequate techniques yielded the final diagnosis of bacterial chronic osteomyelitis of the jaw. Its management requires a multidisciplinary approach, involving oral and maxillofacial surgeons, infectiologists and microbiologists, to limit treatment failure. Antibiotic therapy without surgery for 6 months achieved the complete radiographic resolution of the CBCT (Cone Beam Computed Tomography) and the normalization of laboratory tests. After 2 years of follow-up, no relapse had been reported. Modern microbiological investigation and sampling techniques are critical for the accurate diagnosis and management of osteomyelitis of the jaw, especially in unusual and clinically misleading forms of this infection.

SIALODOCHITIS FIBRINOSA OF KUSSMAUL: A CASE PRESENTATION

<h3>Objective</h3> Sialodochitis fibrinosa of Kussmaul is a rare phenomenon that was first reported in 1879. It is characterized by recurrent major salivary gland swelling in patients with atopic background. Treatment includes antihistamines, systemic glucocorticoids, massage, mucolytic agents, sialendoscopy, and surgery. To date, less than 70 cases have been reported in the literature. <h3>Case Summary</h3> A 42-year-old woman was referred from the emergency room to the Oral Medicine unit at Sheba Medical Center with the chief complaint of severe xerostomia and asphyxia due to submandibular swelling. Her medical history was significant only for asthma. The patient reported facial and submandibular swelling accompanied by severe pain, 3 weeks before her presentation. Two months earlier, she had developed urticaria, which was treated with antihistamines. The patient denied any symptoms associated with xerophthalmia. Complete blood count as well as lipase, IgG4, and angiotensin converting enzyme (ACE) levels were within normal limits. Blood levels of amylase and C-reactive protein were elevated. Virology (hepatitis B virus, cytomegalovirus, hepatitis C virus, human immunodeficiency virus [HIV], measles, mumps) and autoimmune panel were negative. Salivary glands ultrasound failed to demonstrate any pathologic findings. Clinical examination revealed enlarged cervical lymph nodes, bilateral mild swelling posterior to angle of mandible, perioral rash, dry tongue covered with white coating, erythema around the orifices of parotid ducts, and labial minor salivary glands enlargement. There was no secretion upon milking of major salivary glands. After stimulation with citric acid, foamy saliva secretion was noted, and on milking a mucous plug was released followed by clear saliva secretion. Sialography demonstrated dilated parotid gland ducts. No focal lymphocytic infiltration was noticed on minor salivary gland biopsy. Cytology of the mucous plugs demonstrated the presence of eosinophils. In the light of salivary glands swelling, dilated parotid ducts, the presence of eosinophils within the mucous plugs, and atopic background, the diagnosis of sialodochitis fibrinosa of Kussmaul was made. <h3>Conclusions</h3> Sialodochitis fibrinosa should be included in the differential diagnosis of patients presenting with sialadenitis, recurrent swelling of major salivary glands, and atopic background.

Inhibition of Prekallikrein for Hereditary Angioedema

BackgroundHereditary angioedema is characterized by recurrent and unpredictable swellings that are disabling and potentially fatal. Selective inhibition of plasma prekallikrein production by antisense oligonucleotide treatment (donidalorsen) may reduce the frequency of attacks and the burden of disease.MethodsIn this phase 2 trial, we randomly assigned, in a 2:1 ratio, patients with hereditary angioedema with C1 inhibitor deficiency to receive four subcutaneous doses of either donidalorsen (80 mg) or placebo, with one dose administered every 4 weeks. The primary end point was the time-normalized number of investigator-confirmed angioedema attacks per month (attack rate) between week 1 (baseline) and week 17. Secondary end points included quality of life, as measured with the Angioedema Quality of Life Questionnaire (scores range from 0 to 100, with higher scores indicating worse quality of life), and safety.ResultsA total of 20 patients were enrolled, of whom 14 were randomly assigned to receive donidalorsen and 6 to receive placebo. The mean monthly rate of investigator-confirmed angioedema attacks was 0.23 (95% confidence interval [CI], 0.08 to 0.39) among patients receiving donidalorsen and 2.21 (95% CI, 0.58 to 3.85) among patients receiving placebo (mean difference, −90%; 95% CI, −96 to −76; P<0.001). The mean change from baseline to week 17 in the Angioedema Quality of Life Questionnaire score was −26.8 points in the donidalorsen group and −6.2 points in the placebo group (mean difference, −20.7 points; 95% CI, −32.7 to −8.7). The incidence of mild-to-moderate adverse events was 71% among patients receiving donidalorsen and 83% among those receiving placebo.ConclusionsAmong patients with hereditary angioedema, donidalorsen treatment resulted in a significantly lower rate of angioedema attacks than placebo in this small, phase 2 trial. (Funded by Ionis Pharmaceuticals; ISIS 721744-CS2 ClinicalTrials.gov number, NCT04030598.)