Facial Edema Research Articles

Normalcy in Post-operative Period After Bilateral IJV Ligation: A Rare Case Report.

Ligation of bilateral IJV causes interruption of major part of venous drainage from Dural venous sinuses that causes complications such as gross facial edema, increased intracranial pressure, brain compression, coma and even death increasing the morbidity and mortality post-operatively. This case report illustrates a case of normalcy noted in post-operative period in a patient after ligation of bilateral IJV in left lower alveolus cancer.

OA01 A case report of a patient with Type 1 diabetes mellitus and systemic lupus erythematosus: a management dilemma

Abstract Background Systemic lupus erythematosus (SLE) is a chronic autoimmune disease affecting multiple organs. Up to 30% of patients with SLE will develop other autoimmune conditions. A few cases of SLE and Type 1 Diabetes Mellitus (T1DM) have been reported in literature. Corticosteroids which is key in induction therapy increases the risk of hyperglycemia and hypertension. Other immunosuppressive agents such as mycophenolate mofetil used in management of lupus nephritis increase the risk of hyperglycemia. This poses a challenge in the management of SLE in a patient with T1DM. Aim To describe a management dilemma in caring for a child with T1DM and SLE at a tertiary referral teaching hospital in Nairobi, Kenya. Method Retrospective chart review of a child with T1DM and SLE at Aga Khan University Medical College East Africa, Nairobi, Kenya. Results We discuss a 16-year-old male patient with T1DM since nine years of age on insulin and on follow up in the endocrine clinic. He presented with complaints of facial and lower limb swelling of three days and severe frontal headache associated with nausea for one day. He was also noted to have a strong family history of Sjogren’s disease and diabetes. His initial examination revealed hypertension with a blood pressure of 184/118 mmHg, facial and lower limb oedema. He was commenced on labetalol infusion and amlodipine for blood pressure control, and was to continue with his insulin regimen. Initial urinalysis showed proteinuria and hematuria. An impression of hypertensive emergency with nephropathy was made. Rheumatology review was requested. Investigations revealed an ANA titer of > 1:1280; anti-smith and anti dsDNA antibodies were positive; low C3 and low C4. Echo showed pleural effusion. The kidney biopsy showed features of proliferative lupus nephritis class IV with no features of diabetic nephropathy. A diagnosis of SLE was made and management initiated with hydroxychloroquine, methylprednisolone for three days then oral prednisolone and mycophenolate mofetil. During methylprednisolone therapy, the patient was noted to have unrecordably high blood glucose necessitating an insulin infusion for six days and close adjustment of insulin doses by the endocrinologist. Initially, after the 1st dose of methylprednisolone, his blood pressure seemed to improve. During the course of treatment, his blood pressure was elevated and difficult to control despite being on labetalol infusion, amlodipine, enalapril and carvedilol. Adjustment of antihypertensive drug doses and addition of hydralazine were made by the nephrologist, cardiologist and critical care specialist. He was eventually discharged with controlled blood glucose and blood pressure. He is currently doing well and is on follow up in the nephrology, rheumatology and endocrinology clinics. Conclusion Our case report highlights the challenges that arise while managing patients with SLE and other illnesses such as T1DM. It also points out the necessity of multi-disciplinary care. This may be a challenge in many African health facilities due to a low number of specialized doctors.

Use of an educational video with mobile technology for the informed consent process: A randomised controlled trial on patients undergoing surgery for an impacted lower third molar

The purpose of this study was to determine whether audiovisual presentation of consent information would significantly improve patients’ postoperative recall of risks and complications regarding surgical removal of impacted lower third molars compared to the presentation of traditional written consent information. A randomised controlled study on 59 patients undergoing third molar removal was conducted. Patients in the intervention group (n = 30) viewed an educational video on risks and complications related to surgery using mobile tablets. Control-group patients (n = 29) received written information of the risks and complications. Patients’ postoperative recall of potential risks for dysesthesia of the lower lip and tongue, infection, and bleeding along with surgical complications of facial oedema, trismus, and pain were assessed using true-false tests. The effect of audiovisual information on postoperative recall of the risks and complications was determined by comparing accuracy scores between the intervention group and control group using the independent t-test. The intervention group was found to have significantly better recall scores of the potential risks and complications, due to much higher accuracy in their recall of bleeding and dysesthesia of the lower lip and/or tongue, compared to the control group [mean (SD) 4.70 (0.94) vs 3.76 (1.50), p = 0.003]. The use of an educational video played on mobile tablets rather than a written pamphlet may lead to better understanding of the informed consent process in patients.

Long-term outcomes of facial nerve decompression by transmastoid versus middle cranial fossa approach for traumatic facial paralysis

ObjectivesTo evaluate and compare the long-term results of patients who underwent facial nerve decompression surgery with either transmastoid-supralabyrinthine (TMSL) or combined transmastoid- middle cranial fossa (MCF) approach for traumatic facial nerve paralysis. Materials and methodsThis is a single-center retrospective case-control study. The medical records of traumatic facial paralysis patients with House Brackmann (HB) Grade 6 who underwent facial nerve decompression surgery at via either TMSL or MCF approach between January 2011 and December 2017 were reviewed. The patients who had otic capsule involvement and total sensorineural hearing loss, therefore underwent translabyrinthine facial nerve decompression, and the patients follow-up period has not yet reached four years were excluded from the study. Postoperative HB score and hearing status were compared. ResultsEleven patients were operated with MCF approach (group 1), while 9 patients with TMSL approach (group 2). Average age of patients was 20.04 + 15.2 (range:4–47) years. Three (15 %) patients were female, while 17 (85 %) was male. Geniculate ganglion (90 %) was the most affected segment of the facial nerve. Facial nerve edema was observed in all cases, while intraneural hematoma were encountered in 4 (20 %) cases. Statistically significant improvement in median HB scores were reached in both groups, and no significant difference was observed in post-operative HB scores between both techniques. No significant difference in median AC 0,5–4 khZ and BC 0,5–3 kHz thresholds was observed between both techniques. ConclusionEven middle fossa approach is the best surgical technique to explore geniculate ganglion and labyrinthine segment of facial nerve, the functional results of transmastoid supralabrynthine approach, which is not needed craniotomy with low complication rate are as successful as middle fossa approach in selected patients.

Bilateral choroidopathy as an initial presentation in systemic lupus erythematosus

We present a case of bilateral (B/L) choroidopathy as an initial presentation in systemic lupus erythematosus (SLE). A 25-year-old female presented with complaints of swelling, redness, and diminution of vision (DOV) with myopic shift since 10 days. Oral antihistaminics were prescribed, considering food allergy due to history of ingestion of fish. But she developed further DOV with macular and facial edema after 2 months. On detailed investigations and consultation with a nephrologist and a rheumatologist, she was diagnosed to have SLE and treated with immunosuppressants. This case highlights that ophthalmic examination should be carefully done and monitored as it may be the only sign of presentation and disease progression.

Efficacy of Electroacupuncture with Sparse-Dense-Wave on Patients Suffered Acute Facial Paralysis.

To explore the efficacy of electroacupuncture (EA) with sparse-dense wave form on the improvement of facial nerve edema, facial spasm, and repair of nerve injury in patients with acute facial paralysis. This study enrolled 100 patients who were treated for acute facial paralysis in People's Hospital of Dongxihu District from December 2019 to December 2020. They were randomly divided into the control group (continuous wave) and the sparse-dense wave group (sparse-dense wave), following by being intervened by EA with different wave forms. Then the facial disability index (FDI) score, serum levels of immunoglobulin A (IgA), immunoglobulin G (IgG), and immunoglobulin M (IgM) in the two groups were evaluated. The total effectiveness rate (98.00% and 86.00%, respectively; P < 0.05) and the recovery rate of facial spasm (76.00% and 56.00%, respectively; P<0.05) in the sparse-dense wave group was both significantly higher than that of the control group. After treatment, the scores of physical functions of patients in both groups increased (P < 0.05), the scores of social/well-being dysfunctions decreased (P < 0.05). Besides, the levels of serum IgA, IgG, and IgM in both groups decreased (P < 0.05), and the serum levels in the sparse-dense wave group were significantly lower than the control group (P < 0.05). EA intervention with sparse-dense wave form is effective for patients with acute facial paralysis, in that it effectively reduced the occurrence of facial spasm and promoted the improvement of edema and repair of nerve injury.

H12 Royal rashes: a potted dermatology history in the British monarchy

Abstract Skin diseases have affected a significant proportion of the population throughout history, royalty being no exception. On reviewing monarchs over the centuries, underlying health conditions can be gleaned from their reported skin conditions and physical appearance. In the 14th century, King Henry IV seized the throne from his cousin Richard II. Henry IV was debilitated by a skin disease thought to be leprosy or severe psoriasis, believed to be a punishment from God for being the first king to execute an archbishop. He is reported to have developed great pustules and eventually necrotic ulcers. King Henry VIII, famous for his six wives, also suffered with poor health during his reign from 1509 to 1547. By the age of 44 years, he was reportedly significantly obese, suffering from bilateral leg ulcers that became recurrently infected, leaving him unable to walk. The aetiology of the king’s ulceration may encompass a range of potential diagnoses. It is likely to have been as a result of venous disease with stasis dermatitis alongside trauma and obesity. Henry’s daughter Queen Elizabeth I, who reigned from 1558 to 1603, suffered from smallpox in 1562, which left her face scarred. Her physical scars were concealed with early forms of lead-laced makeup. She also reportedly suffered from hair loss. This may have been telogen effluvium secondary to stress, or it is possible that she suffered from female pattern hair loss. Alternatively, the application of lead to her face may have contributed to her alopecia. The following century, Queen Anne, the monarch that unified England and Scotland and took ownership of Gibraltar, is thought to have suffered from systemic lupus erythematosus. Portraits have demonstrated the classic malar rash, facial oedema and joint swellings. She also suffered many miscarriages and eventually a lethal stroke, raising suspicion for associated antiphospholipid syndrome. Moving to the 20th century, Prince William of Gloucester, a grandson of King George V, suffered from a photoexacerbated blistering skin rash. Three years later, he was diagnosed with porphyria, giving credence to the theory that the madness of King George III, one of William’s ancestors, was due to porphyria. However, this has been disputed, and manic–depressive psychosis is felt to be the more likely explanation. With up to 33% of the UK population suffering with skin conditions at any one time, a significant number of the current royal family will have a variety of skin diseases. How will their dermatological history be told in the future?

Unusual cause of unilateral facial oedema: intramuscular dirofilariasis

IntroductionUnilateral facial oedema may occur due to inflammatory, infective, or cystic pathology; patients often seek medical help at an early stage.Case reportWe report one such case caused mimicking a parotid abscess, caused by dirofilariasis.ConclusionDirofilariasis is an emerging zoonosis and should be considered a differential diagnosis of atypical facial swelling. It is equally important for clinicians, radiologists, and pathologists to be familiar with the diagnostic characteristics to avoid misdiagnosis.

Antibiotic-induced DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) in an infant with Down syndrome

IntroductionDrug Rash with Eosinophilia and Systemic Symptoms syndrome (DRESS) is a rare but serious drug reaction. This syndrome is associated with an immune reaction triggered by the use of certain medications. Rare cases of DRESS have been reported in early infancy or in infants with chromosomal disorders, making their prognosis unknown. ObservationA 20-month-old infant with trisomy 21 was hospitalized for a febrile (39°C) rash with systemic symptoms. He had been hospitalized twenty days earlier for pneumonia, which was treated with antibiotics (cefotaxime and vancomycin). Physical examination revealed facial edema, a diffuse maculopapular rash involving the entire body and face and cervical lymphadenopathy. Laboratory tests showed leukocytosis with eosinophilia and liver damage (liver transaminases were 20 times the normal level). The diagnosis of DRESS syndrome due to antibiotics was made based on a RegiSCAR score of 7. The infant was treated with IV corticosteroids at a dose of 1mg/kg/day, along with a 2g/kg infusion of gammaglobulin. Clinical and laboratory improvement was observed with disappearance of skin lesions and normalization of laboratory values. DiscussionThe diagnosis of DRESS syndrome due to antibiotics was made based on a RegiSCAR score of 7. This class of drugs is the leading cause of DRESS syndrome described in children. The evolution of our infant patient with Down syndrome and DRESS syndrome was favorable.

The Impact of Professional Oral Hygiene in Orthognathic Surgery.

Oral hygiene represents a fundamental element with regard to outcomes in oral and maxillofacial surgery. Both basic hygiene that the patient must perform daily at home and obviously professional hygiene aimed at preventing any complications in the surgical field. Patients undergoing orthognathic surgery in our Department of Maxillofacial Surgery in Ancona, in a total number of 137 in the period studied (2016-2021), were divided into 2 groups: period 1, (2016-2018) and period 2 (2019-2021). The division into 2 groups derives from the establishment of a professional oral hygiene protocol established starting from January 2019 and put into practice by dental hygienists. The protocol consists of preoperative counseling and above all very precise instructions in the postoperative so as to optimize the oral hygiene process, assist in the best possible wound healing and reduce possible postoperative complications. Anamnestic, cephalometric data, measurements of the distances between certain landmarks for the evaluation of facial edema, patient-referred pain, accurate intraoral and extraoral physical examination both pre and postoperative, and complications were collected for each patient. The main goal of the study authors set is to evaluate the effective impact of professional oral hygiene in the pre and postoperative management of patients undergoing orthognathic surgery, taking into account parameters, such as facial edema and pain, and using evaluation scales, making a comparison with the data reported in the 3 years before and after the establishment of the protocol applied by the authors.

Efficacy and safety of tunlametinib in patients with advanced NRAS-mutant melanoma: A multicenter, open-label, single-arm, phase 2 study.

9510 Background: NRAS-mutant melanoma is an aggressive subtype with worse prognosis. However, no targeted therapy has been approved to date worldwide. Tunlametinib (HL-085), a novel, potent, selective, oral small-molecule MEK1/2 inhibitor, has showed a favorable pharmacokinetics profile, acceptable tolerability and encouraging antitumor activity in the phase 1 study (BMC Med. 2023 Jan 4;21(1):2). Methods: This is an ongoing, multicenter, open-label, single-arm, phase 2 pivotal registration study. Patients (pts) with NRAS-mutant unresectable stage III or IV melanoma were enrolled and received tunlametinib 12 mg orally twice daily. A historical control of objective response rate (ORR) of 10% was predefined for sample size estimation (100 pts, assuming loss to follow-up rate of 5%) and efficacy evaluation. The primary endpoint was the confirmed ORR per RECIST v1.1 assessed by independent radiology review committee. Results: A total of 100 pts were enrolled. All pts were included in the safety analysis set and 95 pts were included in the full analysis set (FAS) for efficacy analysis. At cut-off date (August 17, 2022), median follow-up was 7.9 months (95% CI: 6.6, 9.8). In the FAS, the median age was 58.0 years (range: 24 to 84). Sixty-four pts (67.4%) had received prior immunotherapy (PD-1/PD-L1 inhibitor). Fifty-six pts (58.9%) were acral melanoma, 16 (16.8%) mucosal melanoma and 12 (12.6%) skin melanoma. Fourteen pts (14.7%) were stage III and 81 (85.3%) stage IV. The most frequent NRAS mutation types were Q61R (40.0%), Q61K (29.5%) and G12D (9.5%). Confirmed ORR was 34.7% (95%CI: 25.3%, 45.2%). Median progression-free survival was 4.2 months (95%CI: 3.5, 5.6), overall survival was immature, and 1-year survival rates was 57.2% (95% CI, 44.7%, 67.8%). Subgroup analysis showed that, in pts who had previously received immunotherapy, the confirmed ORR was 39.1% (95% CI: 27.1%, 52.1%). The most frequent treatment related adverse events (TRAEs) were increased blood creatine phosphokinase (CK), diarrhea, peripheral oedema, facial oedema and increased aspartate aminotransferase. Grade ≥3 TRAEs occurred in 68 pts (68.0%), of which 38.0% (38/100) were increased blood CK. Most of pts with CK elevation are asymptomatic and can be managed by dose interruption or reduction without permanent treatment discontinuation. No treatment-related death was reported. Conclusions: Tunlametinib was well tolerated and demonstrated encouraging treatment response rate in pts with advanced NRAS-mutant melanoma. These results indicate that tunlametinib could be a promising treatment option for NRAS-mutant melanoma, even for those immunotherapy failed pts. Clinical trial information: NCT05217303 .

A phase 1/2a study of T3011, an oncolytic HSV expressing IL-12 and PD-1 antibody, administered via intratumoral (IT) injection as monotherapy in advanced solid tumors.

2520 Background: T3011, a replication-competent, genetically modified, next-generation oncolytic herpes simplex viruses (oHSV), deletes one copy of ICP34.5 and retains ICP47, thus maximizing replication activity and virulence attenuation of the virus. Moreover, T3011 was inserted with biologically active IL-12 and anti-PD-1 antibody genes. Therefore, while lysing tumor cells, T3011 can significantly improve the tumor microenvironment and elicit the body's specific anti-tumor immunity. Methods: An open-label, dose escalation and expansion study of T3011 administered via IT injection as a single agent in patients (pts) with advanced solid tumors. Pts who progressed on or were intolerable to the standard of care were eligible and received T3011 IT therapy Q2W in several dose cohorts (2.5×105 ~ 1×108 PFU/mL). The primary endpoints were safety and tolerability. Key secondary endpoints were PK/PD profile, efficacy endpoints including ORR, DCR, DOR, PFS by investigator per RECIST1.1 and OS. Results: As of 18 Jan. 2023, 90 Pts received T3011 monotherapy. Median follow-up time is 5.7(0.5-22.5) months. No DLT events were reported. Treatment-related adverse events (TRAEs) were observed in 66.7% pts (≥G3 TRAEs and treatment discontinuation both in 2.2% [2/90]), and treatment-related SAEs occurred in 2.2% (2/90) pts. The most frequently reported TRAEs (≥5%) were pyrexia (21.1%, 19/90), influenza like illness (8.9%, 8/90), TSH increased (6.7%, 6/90), proteinuria (6.7%, 6/90), facial edema, white blood cell count decreased, AST increased and anemia (5.6%, 5/90, each). MTD was not reached, RP2D was determined as 1x108 PFU/mL. Over 80% of pts had viral DNA un-detected in their blood, urine and saliva after injection. Moreover, almost all injection site and occlusive dressing samples that tested positive for viral DNA were confirmed to be free of viral activity. The confirmed ORR was 11% (6/55), DCR was 49% (27/55) in 55 pts (HNSCC, sarcoma, melanoma, breast cancer, etc.) treated under RP2D evaluable for tumor response.The median PFS was 2.8 mos (95%CI: 1.84, 4.60) in all 76 Pts received T3011(RP2D). 19 head and neck squamous-cell carcinoma (HNSCC) pts progressed on platinum-based chemotherapy and anti-PD-1/PD-L1 therapy treated with T3011(RP2D), median PFS was 3.9 mos (95%CI: 0.89, NA;1 pt failed from chemotherapy only for PFS analysis set). 12 HNSCC pts had at least one tumor evaluation post-dose, the confirmed ORR and DCR was 25% (3/12) and 50% (6/12) respectively. Conclusions: The safety profile and clinical compliance of T3011 IT therapy are excellent. T3011 injected locally will not spread to blood, urine and saliva, regional shedding doesn't raise the possibility of transmission to the local and systemic environment. The encouraging anti-tumor activity of T3011 monotherapy in advanced HNSCC pts supports further evaluation in phase II studies. Clinical trial information: NCT05602792 .

Combination targeted therapy with avapritinib and sunitinib in patients with refractory gastrointestinal stromal tumors after failure of standard treatments: A small prospective pilot study.

11538 Background: There is currently no standard treatment after ripretinib beyond the fourth-line setting for refractory gastrointestinal stromal tumors (GISTs). A combination of tyrosine kinase inhibitors (TKIs) is rational to be potentially effective after failure of standard therapies. The objective of this study was to explore the efficacy and safety of avapritinib plus sunitinib in refractory GISTs. Methods: This was a prospective cohort study in part of a real-world trial (NCT05461664) exploring avapritinib in GIST patients (pts) after failure of standard treatments. Participants received avapritinib 100–200 mg once a day (QD) combined with sunitinib 25–37.5 mg QD continuously in 28-day cycles until disease progression (PD) or discontinuation from January 2022 to January 2023. Clinical outcomes including objective response rate (ORR), survival and safety were assessed. Results: A total of 11 pts with a median age of 57 years were enrolled. Median follow-up duration was 4.1 months (range, 1.4–12.8 months). Median sum of target lesions was 28.2 cm (range, 6.6–47.0 cm). Four pts had primary KIT exon 11 mutation, 6 had exon 9 and one had exon 17, secondary mutation KIT exon 13 was in 2 pts, exon 14 in one, exon 16 in 2 and exon 17 in 7 pts. Seven pts received more than 4 lines of prior TKIs, three received 3 and one had 2 lines of prior TKIs. One patient (9.1%) achieved partial response (PR) with 48.7% lesion length reduction, 9 (81.8%) had stable disease (SD) with 8 out of 9 pts achieving tumor shrinkage (median sum of lesion reduction of 20.8% [range, 9.8–27.3%]), and one (9.1%) had PD according to mRECIST1.1. Eight pts (72.8%) achieved PR, one (9.1%) had SD, and 2 (18.2%) had PD according to the Choi criteria. Until last follow-up, 4 pts progressed, one withdrew from the study and one discontinued treatment after surgery. Five pts were still receiving treatment, one exceeded 1 year with 26.3% lesion reduction in the seventh line setting. Progression-free survival and overall survival results were immature. ORR and lesion reduction trended favorably in those with KIT activation loop (AL) mutations, but this was not statistically significant. The dose of avapritinib (100–150 mg/d) and sunitinib (25–37.5 mg/d) could be tolerated. Common adverse events (AEs) were anemia, leukopenia, diarrhea, fatigue, periorbital and face oedema, and memory impairment. Common grade ≥3 AEs included anemia (54.5%), leukopenia (36.4%), diarrhea (36.4%), tumor hemorrhage (27.3%) and gastrointestinal hemorrhage (18.2%). Conclusions: The preliminary results from this study demonstrated meaningful clinical benefit with avapritinib plus sunitinib, achieving significant lesion reduction in refractory GISTs, especial those with KIT AL secondary mutations. Further studies are warranted to determine the optimal dosing regimen of two targeted drugs. Clinical trial information: NCT05461664 .

Una “vistosa” vasculite con edema, che riguarda gli arti e il viso e risparmia il tronco

Acute haemorrhagic oedema of infancy (AHEI) is a small vessel vasculitis that affects boys between 4 months and 2 years. It presents with a characteristic purpuric skin rash but with marked bruising and “typical” localizations in auricles and is often accompanied by oedema of face and extremities. It is typically post-infectious, especially after infections of the upper respiratory tract and rarely can be associated with gastrointestinal, renal, articular or testicular complications. The paper reports the case of a 2-year-and-5-month-old boy who, 5 days after the resolution of a febrile infection of the upper airways (with subsequent detection of positive IgM and IgG serology for SARS-Cov-2), presented with a rash with ecchymotic/purpuric lesions in his lower limbs, face and auricles associated with intense arthralgia, arthritis of a knee and deterioration of general conditions. The diagnosis of acute infantile haemorrhagic oedema was clear but the clinical presentation required an “important” therapeutic approach with intravenous steroids and an infusion of immunoglobulins.

Comparison of Manual (2D) and Digital (3D) Methods in the Assessment of Simulated Facial Edema

An edema assessment following dental surgeries is essential to improving the dental surgeon's technique and, consequentially, patient comfort. Two-dimensional (2D) methods are limited in analyzing 3-dimensional (3D) surfaces. Currently, 3D methods are used to investigate postoperative swelling. However, there are no studies that directly compare 2D and 3D methods. The goal of this study is to directly compare 2D and 3D methods used in the assessment of postoperative edema. The investigators implemented a prospective, cross-sectional study with each subject serving as its own control. The sample was composed of dental student volunteers without facial deformities. The predictor variable is the method used to measure edema. After simulating edema, manual (2D) and digital (3D) techniques were used to measure edema. The manual method used direct facial perimeter measurements. The two digital methods were photogrammetry using a smartphone (iPhone 11, Apple Inc, Cupertino, California), and facial scanning with a smartphone application (Bellus3D FaceApp, Bellus3D Inc, Campbell, California) [3D measurements] MAIN OUTCOME VARIABLE: The coefficient of variation (CV) (CV=standard deviation /mean) was used to assess homogeneity of edema measurements. The Shapiro-Wilk and equal variance tests were applied to assess data homogeneity. Next, one-way analysis of variance was performed, followed by a correlation analysis. Finally, the data were submitted to Tukey's test. The statistical significance threshold was set at 5% (P<.05). The sample was composed of 20 subjects aged 18-38years. The CV showed higher values using the manual (2D) method (47%; 4.88%±2.99), compared with the photogrammetry method (18%; 8.55mm±1.52) and the smartphone application (21%; 8.97mm±1.93). A statistically significant difference was observed between the manual method values and those of the other two groups (P<.001). There was no difference between the facial scanning and photogrammetry groups (3D methods) (P=.778) CONCLUSION AND RELEVANCE: Both digital measuring methods (3D) demonstrated greater homogeneity than the manual method in analyzing facial distortions caused by the same swelling simulation. Therefore, it can be affirmed that digital methods may be more reliable that manual methods for assessing facial edema.

Clinicoprognostic Study of Hydroa Vacciniforme-like Lymphoproliferative Diseases: A Systematic Review

Background: Limited clinicopathological and prognostic data are available on hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD). Methods: This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020. Results: A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality. Conclusion: HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.

A Case of Peripheral T-Cell Lymphoma Mixed With Invasive Fungal Sinusitis of Maxillary Sinus

A Non-Hodgkin’s lymphoma rapidly forms a deep ulcer and invades the sinuses, orbital wall, and the palate. It is important to detect and treat early as it can, although rarely, be accompanied by rapid ulcerative destruction and sepsis due to necrosis. An 80-year-old female visited our hospital complaining of right nasal congestion and facial edema. A fungal ball in the right maxillary sinus and a defect in the anterior wall of the maxilla were observed by paranasal sinus computed tomography, and sinus endoscopic surgery and Caldwell Luc operation were performed. The intraoperative biopsy showed findings suitable for invasive fungal sinusitis. Based on this, a secondary medial maxillectomy was performed; however, peripheral T-cell lymphoma mixed with invasive fungal sinusitis was reported on the permanent biopsy. We report a case of peripheral T-cell lymphoma mixed with invasive fungal sinusitis of maxillary sinus.

P92 PERIMYOCARDITIS AS FIRST MANIFESTATION OF DYSTHYROIDISM: AN UNUSUAL CASE

Abstract Thyroid and cardiovascular system are inextricably related and cardiovascular complications can occur in both subclinical and overt thyroid dysfunction. We report the case of a 42–year–old woman presenting with chest pain. She had a positive SARS–CoV–2 swab in June and she was hospitalised in suspicion of COVID–related pneumonia and pericarditis in July: treatment with ibuprofen and colchicine was started and patient was discharged. On July 26th, the patient came back to emergency department due to worsening chest pain and myalgias after therapy decalage. She had face edema and hoarse voice. 12–leads EKG was normal; echocardiography showed 8 mm pericardial effusion and normal ejection fraction. Blood testing revealed no inflammatory signs, a mild increase of Troponin (peak value 19.1 ng/L, n.v. 0–14) and a high value of CPK (peak value 2299 U/L, 464 U/L at time of discharge, n.v. 30–150). Moreover, she had a biohumoral profile of Hashimoto thyroiditis: severe hypothyroidism with increase in TSH levels (538 microU/ml peak value, 322 microU/ml at time of discharge, n.v. 0.27–4.2) and positivity to thyroid autoantibodies. The patient underwent a cardiac magnetic resonance imaging that was suggestive for acute perimyocarditis with biventricular diffuse myocardial edema, preserved biventricular function and mild–to–moderate pericardial effusion. Low dose L–T4 therapy was started together with liothyronine that was stopped after few days, once acceptable FT3 concentrations were obtained. At echocardiography control after 8 days, we registered a significant reduction of pericardial effusion (2 mm). Myocarditis is an inflammatory disease of the myocardium and it is predominantly mediated by viral infection. A rare cause of myocarditis is represented by dysthyroidism. In our case, the severe hypothyroidism seems to be the cause of myocardial and pericardial edema. The failure in diagnosis of this endocrinological disorder can determine a delay in specific therapy with possible serious cardiovascular manifestations. So, it would be reasonable to check the thyroid status of all patients presenting with myopericarditis, in the absence of another clearly identifiable aetiology. The role of to the prior SARS–CoV–2 infection in not certain in our case, but the quick recovery after specific endocrinological therapy suggests the low probability of perimyocarditis as SARS–CoV2 related manifestation.

Non-infectious skin manifestations in patients with Chronic Granulomatous Disease: A report of 4 cases

BackgroundChronic granulomatous disease (CGD) is a monogenic inborn error of immunity caused by defects in the phagocytic NADPH oxidase complex. These defects lead to an increased risk of severe infection, autoimmunity, widespread granuloma formation and dysregulated inflammation. In individuals with CGD, immune dysregulation and autoimmune manifestations may impact almost every organ system with atypical dermatologic findings frequently reported. Case PresentationsHere we describe four cases of inflammatory skin lesions in patients with genetically confirmed CGD. Patient A is a 4-year-old male who presented with diffuse alopecia totalis preceded by a widespread skin eruption composed of polycyclic, annular, erythematous plaques and prominent facial erythema and edema. Whole exome sequencing was obtained due to concern for ectodermal dysplasia, which identified a variant in CYBB (CYBB c.901G>T; pVal301Phe). Patient B is a 4-year-old male with X-CGD (CYBB c.1016 C > A, p.Pro339His) who developed annular to polycyclic indurated red plaques on his face and arms. Patient C is a 19-year-old female with autosomal recessive CGD (NCF1 c.75_76delGT) and persistent hidradenitis suppurativa-like lesions in the axilla, inguinal areas, and popliteal fossa. Patient D is a 12-year-old female with autosomal recessive CGD (p47phox deficiency) and psoriasiform dermatitis of the scalp.Patient A had persistence of lesions that required chronic systemic corticosteroids and methotrexate. He underwent hematopoietic stem cell transplantation using a matched sibling donor with improvement in his dermatitis. Skin lesions in patient B resolved with the use of topical corticosteroids. Patient C improved with a combination of isotretinoin therapy and both intralesional and systemic corticosteroid treatment. Patient D had improvement in her psoriasiform dermatitis with topical corticosteroid solution and salicylic acid shampoo. ConclusionsThis case series expands our understanding of cutaneous non-infectious eruptions that can occur in patients with both X-linked and autosomal recessive forms of CGD. Each of these cases illustrates a unique clinical history, histologic findings and treatment plan in patients who developed cutaneous lesions secondary to immune dysregulation in the context of CGD. This series provides additional insights that may assist providers in identifying and managing non-infectious dermatologic manifestations of CGD.

Solid Facial Edema: A Rare Complication in Acne

Solid persistent facial edema is a rare and poorly understood condition that can be a serious complication of acne vulgaris and rosacea. It manifests as a slow onset of persistent lymphedema in the upper two-thirds of the face and can lead to significant cosmetic disfigurement affecting a patient's self-esteem and mental health. This article presents a case of persistent facial edema in a patient with severe acne and discusses the diagnosis and management of this rare condition.