Recurrent Abscesses Research Articles

Cavernosal abscess and primary scrotal calcinosis in the diabetic patient: A case report and review of literature

Abstract A cavernosal abscess is a rare urological condition to occur. It is classified as primary and secondary. The primary is mostly idiopathic, whereas the secondary is associated with some precipitating factors. Most of the cases were diagnosed clinically. In some cases, imaging such as an ultrasound of the local region or magnetic resonance imaging may be required. Cavernosal abscesses can be treated using a minimally invasive procedure such as image-guided drainage or incision and drainage along with systemic antibiotics. A 52-year-old uncircumscribed uncontrolled diabetic patient presented with a complaint of swelling in the penile region for the 1-week duration. He also complains of pain in the penile region for a 5-day duration. He is a known case of type 2 diabetes on oral hypoglycemic drugs for the past 2 years. Clinical examination confirmed the diagnosis of cavernosal abscess. The patient underwent circumcision with incision and drainage of abscess under higher antibiotic coverage (vancomycin and metronidazole). The patient underwent local excision of primary scrotal calcinosis after 1 month. The patient is followed up for more than 2 years without any evidence of recurrence or impotence. The cavernosal abscess is one of the rare urological conditions, which needs immediate surgical intervention to reduce future complications such as erectile dysfunction and recurrent abscess. Uncontrolled diabetes has a high chance of developing cavernosal abscesses even without precipitating factors.

Diagnosis of a case of X-linked agammaglobulinemia with juvenile idiopathic arthritis and recurrent pneumonia in Bangladesh

Abstract X-linked agammaglobulinemia (XLA) is a rare hereditary primary immunodeficiency disorder caused by mutation in the Bruton’s tyrosine kinase (BTK) gene located in the Xq22 region of the X chromosome. It is characterized by absolute or marked deficiency of matured B-cells in circulation as well as decreased in all immunoglobulin (Ig) classes. We have reported one case who is a 48-month-old boy and suffering from recurrent pneumonia, skin abscess, otitis media, and swelling of the left knee joint. His serum Ig levels showed an IgM level of <0.169 g/l, IgG 2.43 g/l, and IgA <0.256 g/l. Immunophenotyping of lymphocyte subsets showed an absence of B-cells. Intracellular BTK protein expression in monocytes by flow cytometry showed a markedly reduced mean fluorescence index which confirmed the diagnosis of XLA. Antibiotics with intravenous Ig therapy were started.

Rates and Predictors of Long-term Clinical Outcomes in Patients With Perianal Crohn's Disease on Biologic Therapy.

Perianal Crohn's disease (pCD) represents an aggressive phenotype with limited studies on long-term outcomes. We evaluated 5-year outcomes of these patients on biologic therapies. We performed a retrospective analysis of patients with pCD at a tertiary medical center. We used Kaplan-Meier curves to estimate rates and multivariate logistic regression to identify predictors of long-term outcomes. We included 311 patients with pCD of which 168 patients were started on biologics [138 anti-tumor necrosis factor (TNF) α, 14 vedolizumab, 16 ustekinumab] at the time of diagnosis. Anti-TNF use at the time of diagnosis was associated with decreased rates of perianal abscess recurrence [hazard ratio (HR)=0.48, 95% confidence interval (CI): 0.32-0.74], whereas ustekinumab use was associated with increased rates of perianal fistula closure (HR=3.58, 95% CI: 1.04-12.35) and decreased rates of perianal abscess recurrence (HR=0.20, 95% CI: 0.07-0.56) at follow-up. Among patients who failed their first anti-TNF, switching to another anti-TNF was associated with decreased rates of colectomy (HR=0.20, 95% CI: 0.04-0.90) and permanent diversion (HR=0.16, 95% CI: 0.03-0.94) compared with ustekinumab, whereas vedolizumab use was associated with decreased perianal fistula closure (HR=0.22, 95% CI: 0.05-0.96) compared with ustekinumab. Predictors of colectomy included colonic disease (odds ratio=2.71, 95% CI: 1.36-5.38) and anal stenosis (odds ratio=4.44, 95% CI: 1.59-12.43). Type of biologic use at the time of pCD diagnosis or after first anti-TNF failure may be associated with long-term outcomes in patients with pCD.

Watchful Waiting After Radiological Guided Drainage of Intra-abdominal Abscess in Patients With Crohn's Disease Might Be Associated With Increased Rates of Stoma Construction.

Management of spontaneous intra-abdominal abscess (IAA) in patients with Crohn's disease (CD) with radiologically guided percutaneous drainage (PD) was debated. This is a secondary analysis from a multicenter, retrospective cohort study of all the patients with CD who underwent PD followed by surgery at 19 international tertiary centers. Seventeen patients (4.8%) who did not undergo surgery after PD were compared to those who had PD followed by surgical intervention 335/352 (95.2%). Patients who had PD without surgery were those with longer disease duration, more frequently had previous surgery for CD (laparotomies/laparoscopies), enteric fistula, on steroid treatment before and continue to have it after PD. Patients who had PD without subsequent surgical resection had a higher risk of stoma construction at later stages 8/17 (47.1%) versus 90/326 (27.6%) (P < .01). Patients with PD with no subsequent surgery had numerically higher rates of abscess recurrence 5/17 (29.4%) compared to those who had PD followed by surgery 45/335 (13.4%) the difference was not statistically significant (P = .07). Even with the low number of patients enrolled in this study who had PD of IAA without subsequent surgery, the findings indicate a markedly worse prognosis in terms of recurrence, length of stay, readmission, and stoma construction. Watchful waiting after PD to treat patients with spontaneous IAA might be indicated in selected patients with poor health status or poor prognostic factors.

Candida Prostatitis: A Review and Update

Candidiasis is a terminology that is used for a fungal infection that is caused by a yeast which is a fungus that called candida species. Some species of candida can cause infection in human beings and the commonest candida species that can infect human beings is candida Albicans. Candida normally lives upon the skin as well as inside the human body, such as the mouth, the throat, the gut, and the vagina, without causing problems. Candida can cause infections if it grows out of control or if it enters deep into the body. For example, it could cause infections in the bloodstream or internal organs like the kidney, the heart, or the brain. Candida infection can be localized to one body organ or it could be a disseminated candida infection affecting various organs. Candida infection does tend to afflict immune-supressed individuals more often in comparison with immune competent individuals. Candida prostatitis an uncommon condition which clinicians should have a high index of suspicion for because it does manifest with non-specific symptoms that tend to be associated with more common conditions of the prostate and the urinary tract. Candida infection of the prostate gland may be an isolated de novo infection or it may be associated disseminated candida infection, or it may also be associated with prostate cancer or various immune-suppression conditions. Candida infection of the prostate gland could manifest as an acute infection/inflammation of the prostate gland or a chronic inflammation of the prostate gland or it could manifest as prostatic abscess. Some of the potential manifestations of candida prostatitis and or prostatic abscess include: (a) incidental finding upon biopsy of the prostate gland or following trans-urethral resection of prostate gland or prostatectomy. ( b) patient may manifest with lower urinary tract symptoms, (c) a patient may manifest with urinary retention that may be acute or chronic, (d) a patient may manifest with raised levels of serum prostate specific antigen (PSA), € at times when digital rectal examination is undertaken on a patient who has Candida prostatitis, the prostate gland may feel benign and in the case of a candida prostatic abscess rectal examination may demonstrate bogginess in the area of the prostate with soft fluctuant feeling. (f) Eosinophil count in some cases of Candida prostatitis would tend to be normal but in some cases of candida infection of the prostate gland, there could be Eosinophilia but this would not be diagnostic of Candida infection, (g) a history of having had coital contact with an individual who has been treated for candida infection or a history of past treatment of the individual should alert all clinicians to exclude the possibility of candida prostatitis. (h) on rare occasions urine culture or culture of expressed prostatic secretions would yield a growth of Candida. Diagnosis of Candida infection or abscess of the prostate gland may be confirmed by positive culture of Candida in prostate biopsy specimen or resected or excised prostate specimen. Treatment of Candida Prostatitis / prostatic abscess does tend to entail: (a) Treatment with appropriate antifungal medicament, plus (b) Complete radiology image-guided aspiration / drainage of any abscess seen plus / minus or endoscopic deroofing trans-urethral resection of the prostate to ensure the abscess drains out completely. Because recurrence of Candida prostatitis or prostatic abscess or Candida infection elsewhere can occur, it is important for patients to have regular follow-up assessments to ensure recurrence disease does not develop and if it develops, it is diagnosed quickly in order to initiate prompt treatment. It is also important to assess all coital contacts of the patient to ascertain if they have Candida infection to enable prompt treatment of their infection. If an individual who has candida prostatitis or prostatic abscess is also found to have contemporaneous adenocarcinoma of the prostate, the carcinoma of the prostate gland should be treated appropriately based upon the Gleason Grade and the Stage of the carcinoma, the performance status and age of the individual patient based upon the national and international guidelines pertaining to prostate cancer as a separate multi-disciplinary team discussion of the patient management.

P104 Use of intravenous antibiotics in severe hidradenitis suppurativa: a multicentre retrospective study

Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory condition, characterized by recurrent abscesses and sinus tracts in intertriginous areas. Intravenous (IV) antibiotics are used in cases of severe HS after the failure of conventional therapies, or to optimize patients prior to initiation of biologics or surgery. However, there is limited evidence on the efficacy of IV antibiotics [Join-Lambert O, Coignard-Biehler H, Jais J-P et al. Efficacy of ertapenem in severe hidradenitis suppurativa: a pilot study in a cohort of 30 consecutive patients. J Antimicrob Chemother 2016; 71:513–20; Ingram JR, Collier F, Brown D et al. British Association of Dermatologists guidelines for the management of hidradenitis suppurativa (acne inversa) 2018. Br J Dermatol 2019; 180:1009–17]. Our aim was to determine the immediate and long-term impact of IV antibiotics in severe HS and to develop a pre- and post-IV antibiotics assessment and care pathway. We conducted a multicentre retrospective review of patients with HS treated with IV antibiotics at two tertiary HS centres between 2013 and 2022. In total, we included 30 patients (14 females and 16 males), with a mean (SD) age of 42.6 (12.8) years (range 15–66) and a mean (SD) body mass index of 29.96 (7.31) kg m−2. All patients were classified as Hurley stage 3. The majority of patients received ertapenem (n = 24); other antibiotics included ceftriaxone (n = 5) and meropenem (n = 1). Patients had an average of 38.7 days on treatment (range 14–56). There were statistically significant improvements at the completion of antibiotics in biochemical and haematological parameters, including C-reactive protein −27.5 mg L−1 [95% confidence interval (CI) −11 to −57; P &amp;lt; 0.003], white cell counts −1.75 × 109 cells L−1 (95% CI −0.95 to −2.80; P &amp;lt; 0.001) and haemoglobin +7 g L−1 (95% CI 2.5–13; P &amp;lt; 0.005). Dermatology Life Quality Index (out of 30) also improved significantly at completion (−5, 95% CI −1 to −10; P &amp;lt; 0.008). These were all maintained 12 months postcompletion. Clinical severity of HS assessed through pain visual analogue score (VAS), discharge VAS, DERMVAS, overall VAS, and HS Physician Global Assessment showed improvement but did not reach statistical significance. Improvement in discharge VAS [out of 10; −2, 95% CI −1.5 to −4.5 (P &amp;lt; 0.066)] achieved borderline significance. To our knowledge, this is the largest analysis of the efficacy of IV antibiotics in patients with the most severe form of HS (i.e. Hurley stage 3), where we show improvement in clinical parameters and quality of life. The findings of this study should encourage a randomized controlled study to determine conclusive evidence to guide the evidence-based management of severe HS and have informed the development of a more formal assessment pathway.

P90 Adalimumab treatment for hidradenitis suppurativa: a retrospective analysis of demographics, patient characteristics and treatment outcomes for 107 patients from a tertiary dermatology centre

Abstract Hidradenitis suppurativa (HS) is a chronic inflammatory disease characterized by recurrent painful nodules, abscesses and sinus tracts in predominantly apocrine gland-bearing skin. Adalimumab is an antitumour necrosis factor biologic approved by the National Institute for Health and Care Excellence in June 2016 for the management of moderate-to-severe HS and is the only licensed biologic for HS. We conducted a retrospective review of patients attending our specialist HS service who were initiated on adalimumab. Demographics, Hurley stage, HS physician global assessment (HS-PGA), patient-reported psychosocial outcomes and reasons for treatment discontinuation were examined. The cohort comprised 57% females (53%). Seventy-four per cent of patients were overweight or obese (n = 80), with a mean weight of 90 kg. Seventy-eight per cent were current or ex-smokers (n = 83). Twelve per cent reported a positive family history of HS (n = 13). Patients had an average baseline Hurley score of 2.8, representing moderate-to-severe disease (Hurley stages 2–3) and a HS-PGA of 3.7 (3 = moderate and 4 = severe). Concomitant treatment with antibiotics and/or systemic therapy was required in 44% of patients (n = 48). Oral antibiotics (n = 30; 28%), Septrin® (n = 16; 15%) and acitretin (n = 10; 9%) were the three most commonly coprescribed medicines. Nineteen per cent of patients underwent a surgical procedure for HS during treatment. The average treatment duration of adalimumab was 22 months. Thirty-two per cent of patients discontinued. Reason(s) for discontinuation included primary failure (n = 13), side-effects (n = 12), malignancy (n = 3; lung adenocarcinoma, plasma cell myeloma, extramammary Paget disease), COVID-19 concerns (n = 3), loss to follow-up (n = 2), secondary failure (n = 2), personal stress (n = 1), serious infection (n = 1; tuberculosis) and pregnancy (n = 1). Statistically significant improvements in HS-PGA score at 16 weeks were demonstrated compared with baseline (3.7 vs. 2.8; P &amp;lt; 0.001). Hurley score remained stable at these timepoints. Disease-related quality of life (Dermatology Life Quality Index) also significantly improved from 18.35 at baseline (reflecting severe impact) to 12.4 at 16 weeks (P &amp;lt; 0.001). Statistically significant differences were also found for the following patient-reported outcomes at 16 weeks compared with baseline (rated out of 100): severity (67 vs. 52; P &amp;lt; 0.013), odour (59 vs. 49; P = 0.008), discharge (64 vs. 46; P &amp;lt; 0.001), pain (65 vs. 46; P &amp;lt; 0.001) and fatigue (66 vs. 54; P = 0.006). Paired t-tests were used for all statistical analyses. In summary, adalimumab is an effective treatment for moderate-to-severe HS leading to clinical improvement in the HS-PGA scale and patient-reported outcomes. One-third of patients discontinued treatment due to a lack of response or side-effects. Adalimumab is often used in combination with other systemic therapies and surgery, reflecting the challenges of HS management.

What predicts the recurrence in ıdiopathic granulomatous mastitis?

Idiopathic granulomatous mastitis (IGM) is a rarely seen chronic and benign disease of the breast. IGM usually emerges in women between 30 and 45years of age and within the first 5years after lactation. There is no consensus on the treatment of the disease. Steroids, immunosuppressive agents such as methotrexate and azathioprine, antibiotics, and surgical and conservative treatments can be preferred. In the present study, it was aimed to demonstrate the treatment options and follow-up data of the patients with IGM and to investigate the effective factors on recurrence if developed in the follow-up period. The data of 120 patients diagnosed with idiopathic granulomatous mastitis were evaluated for this cross-sectional retrospective study. The demographic, clinical, treatment, and follow-up features of the patients were obtained from the file records. The median age value of the 120 female patients included in the study was 35 (24-67) years. Of the patients, 45%, 79.2%, 49.2%, and 15% had a past history of surgical intervention, steroid use, methotrexate use, and azathioprine use, respectively. Recurrent lesion developed after the treatment in 57 (47.5%) patients. The recurrence rate was 66.1% in the patients who underwent surgical intervention in the initial treatment. There was a statistically significant difference between the patients with and without recurrence regarding the presence of abscess, the presence of recurrent abscess, and having surgical intervention as the initial treatment in the past history. The rate of having surgery was statistically significantly higher compared with the administration of steroid therapy alone and the combination of steroid and immunosuppressive therapy in the initial treatment of the patients who developed recurrence. The rate of having surgery together with the administration of steroid and immunosuppressive therapy was statistically significantly higher than the administration of steroid and immunosuppressive therapies. Our study showed that surgical intervention and the presence of abscess increased recurrence in the treatment of IGM. Key Points • This study has shown that surgical intervention and the presence of abscess increase recurrence. • A multidisciplinary approach to the treatment of IGM and management of the disease by the rheumatologists may be critical.

ENDOSCOPIC RESECTION OF RECTAL POLYP REVEALING A LEIOMYOMA

Background: Rectal leiomyoma is a rare histological entity withnonspecific appearance on endoscopy and scarce literature regarding its management.The paucity of available literature is likely due to its benign behavior, and treatment is only indicated for symptomcontrol. As such, there are manyunknowns regarding polypectomyeffectiveness as a treatment and whether it is necessary to monitor after excision.Here, we review published cases of rectal leiomyoma and itsproposed endoscopic treatments and outcomes. Case Summary: We report a case of a rectal leiomyoma diagnosed after endoscopic resection of a rectal sessile polyp. A 70-year-old woman with longstanding diabetes was referred for further investigation of chronic constipation and recurrent anal abscess. Colonoscopy revealed a 7-mm sessile polyp in the rectum. Polypectomy was performed using a cold snare, and histology confirmed a rectal leiomyoma and an R0 resection. No bleeding and no residual local lesions were found on control endoscopy. Conclusion: R0 endoscopic resection is an effective, low-risk treatment for rectal leiomyoma, and no follow up is required.

Reconstruction challenges for complicated preauricular sinuses: case reports

Preauricular sinuses and pits are congenital anomalies located in or just in front of the ascending limb of the helix. Even though excising a sinus is a relatively minor surgical procedure, recurrence is not uncommon if an adequate resection is not done. Surgery is only indicated when it is complicated by recurrent infection or abscesses. Simple closure may not be possible when there is large tissue defect. In those circumstances local flaps will be required to cover the defect. In this article we report 2 cases which had large tissue defect after excision and closed with local rotational flaps. This provided good cosmetic outlook and no recurrences so far. There are not enough articles published so far with such kind of reconstruction techniques for preauricular sinus excision tissue defects.

(549) Case Report of an IPP Reservoir Salvage Following Infection

Abstract Introduction Inflatable penile prosthesis (IPP) infections are rare and devastating. IPP infections are treated with either explant or salvage, the latter with either a malleable or inflatable device. Isolated component infections are very rare in the literature. This is the first description of successful treatment of an isolated IPP reservoir infection, with preservation and eventual reuse of the patient’s uninfected pump and cylinders. Objective To report a case of successful IPP salvage after an isolated reservoir infection. Methods This is a case study of IPP reservoir explant and eventual reimplant in a 51-year-old male, with preservation of the implant pump and cylinders. Past medical history is notable for HIV, hypertension, and hyperlipidemia. The patient had a Coloplast Titan IPP placed penoscrotally in November 2020 for treatment of post-prostatectomy erectile dysfunction. The reservoir was placed ectopically in the patient’s right high submuscular space. Results In January 2021 the patient underwent emergent exploratory laparoscopic washout and drain placement at a different hospital for perforated appendicitis. The IPP reservoir was not encountered during this procedure. In March 2021 he presented to that same hospital with recurrent fevers and right lower quadrant pain. CT showed increased peritoneal stranding and thickening surrounding the IPP reservoir as well as multiple small, complex loculated fluid collections suspicious for abscesses external to the reservoir. There was no evidence of infection of the pump or cylinders on imaging or physical exam. He was transferred to Dartmouth-Hitchcock Medical Center for further care. Conservative treatment with intravenous vancomycin, Zosyn, and fluconazole yielded no change after 48 hours. Three days after initial presentation, the reservoir was explanted during a collaborative procedure with general surgery, during which the appendix remnant was also removed. Intraoperatively the penile prosthesis reservoir was encased in a dense rind that was incised and drained. Copious purulent material was sent for culture, which showed multi-organism aerobic and anerobic bacteria. The reservoir was removed and the remnant tubing to the pump was clamped. The abdomen was irrigated repeatedly with saline, dilute betadine, vancomycin, gentamicin, Zosyn, and amphotericin. The prothesis tubing was cut as low as possible, capped, and wrapped with a paste of vancomycin mixed with Zosyn and amphotericin. There were no intraoperative complications. The patient was discharged two days later. At his one-week postoperative visit, he was readmitted for interventional radiology drain placement due to abdominal abscess recurrence. Once the recurrent abscess had resolved, he underwent successful radiation treatment for recurrent prostate cancer. In March 2022, the reservoir was successfully replaced into the left high submuscular space via counter incision and the dormant cylinders and pump were reconnected. The patient continues to do well, with no signs of recurrent infection and with excellent device function. He is satisfied with the result of surgery. Conclusions To our knowledge this is the only case of isolated IPP reservoir infection successfully treated with reservoir explant and eventual replacement. The original IPP pump and cylinders were preserved without issue and eventually reused. Disclosure Any of the authors act as a consultant, employee or shareholder of an industry for: Coloplast.

Outcomes of surgical treatment with patterns of bacterial culture and antimicrobial susceptibility testing in cases of cervical abscessation in dogs: 82 cases (2018–2021)

Objective: The objective of this study was to summarize the presentation, diagnosis, and outcome for dogs surgically treated for chronic cervical abscessation following suspected or reported cervical or oropharyngeal trauma, as well as to report on culture results and antimicrobial susceptibility patterns.Results: Eighty-two dogs were identified by retrospective review. Successful surgical outcome was achieved in 92.7% of dogs. Abscess recurrence was confirmed or suspected in 6/82 (7.3%) cases, and surgical intervention for abscess recurrence was performed in 4/82 (4.9%) cases. Foreign material was identified at surgery in 5/82 (6%) cases. Incisional healing complications were noted in 9/82 (10.9%) cases and required additional surgery in 5/82 (6%) cases. Twenty-three (28%) dogs had negative culture results. The results of antimicrobial sensitivity testing led to a change in antimicrobial treatment in only 9% of cases.Surgically treated cervical abscessation carries a good prognosis with a low incidence of recurrence in this cohort (in contrast to previous reports), despite low frequency of foreign body removal or identification of the underlying cause of the abscess. Excision of chronic inflammatory tissue may not be necessary for a successful outcome, contrary to previous recommendations. Multi-pathogen infections and anaerobic infections are commonly encountered.

Terapeutic Remission of Community Acquired Pneumonia Caused by Rothia Mucilaginosa in an Adult Patient with Idiopathic Pulmonar Fibrosis

Rothia Mucilaginosa (RM) is part of the microbiota of the pharynx and upper respiratory tract. RM is a gram-positive, enveloped, coagulase-negative coccus of the family Micrococcaceae. Clinical manifestations range from mild bronchitis to recurrent pneumonia and lung abscess. It is difficult to determine the clinical significance of this organism in respiratory specimens, therefore, in the diagnosis of pneumonia caused by R. mucilaginosa, bronchoscopy specimens should be cultured. Risk factors associated with Rothia are hematologic malignancies and neutropenia. At present, the importance of Rothia in various pathologies remains unclear due to its difficulty in culture and high potential for contamination at the time of sampling. Although it is an oral microbiota, it has been implicated in pneumonia after being identified in sputum or bronchoalveolar lavage smears and in infectious lesions of the respiratory tract, such as lung abscess and empyema. The challenge of MRI infection in clinical practice is deciding whether it is a real infection or a product of sample contamination. We present the case of a 50-year-old adult patient, without neutropenia, without hematological malignancy, with progressive idiopathic pulmonary fibrosis (IPF) of 1 year of evolution. On admission to the hospital, this person presented signs of respiratory failure, tachycardia, upper rales. auscultation and 85% oxygen saturation, that's why he went through many different studies to diagnose Rothia Mucilaginosa pneumonia.

P-004 APPENDICITIS CAUSING CHRONIC MESH INFECTION: A CASE SERIES OF TWO PATIENTS

Abstract Aim To discuss appendicitis as a cause of chronic mesh infection. Methods This study reports two patients with previous inguinal hernia repair who presented with recurrent groin infections resulting from appendicitis. Results Patient 1 had a history of laparoscopic bilateral inguinal hernia repair, followed by an open repair of a unilateral recurrence. He presented twice with groin pain and discharge. Sinugram demonstrated a superficial tract, but intra-operatively the sinus tract extended to the deep ring where the appendix was adherent and inflamed. There was also a meshoma of the preperitoneal mesh. He underwent appendicectomy and excision of meshoma. Patient 2 presented with perforated appendicitis with a large pelvic collection extending into the anterior abdominal wall. This was treated with percutaneous drainage and antibiotics. Further presentations with recurrent abscesses prompted a laparoscopy where an inflamed appendix was found adherent to the previous mesh through a hole in the peritoneum in a pre-peritoneal abscess cavity. He underwent appendicectomy and washout without mesh removal. Both patients remain symptom free at 10 months and 1 month respectively. Conclusion Literature review found reports of mesh-related appendicitis but no cases of appendicitis causing chronic mesh infection. Whilst rare, appendicitis causing chronic mesh infection should be considered in patients presenting with recurrent groin pain and abscesses having previously undergone inguinal hernia repair. Whilst we do not have long term follow up data, our experience indicates that these patients can be managed with or without mesh removal, providing the appendix is removed and the source of sepsis controlled.

Abnormal Dihydrorhodamine Result in a Patient with Diabetes Mellitus, Recurrent Candida Liver Abscesses: Diagnostic Possibilities

A 17-year-old female with type I diabetes mellitus (DM) and a history of recurrent candidal vaginal infections and liver abscesses was assessed fora potential neutrophil oxidative burst defect by DHR flow cytometry. The DHR result was abnormal with significantly decreased MFI (MFI = 9; ref. cutoff: >/ = 60) after stimulation with PMA though all of her neutrophils (99%; ref. cutoff >/ = 95%) showed respiratory burst. This DHR pattern has been previously observed in patients with complete myeloperoxidase deficiency (cMPO), p40phox deficiency and hypomorphic X-linked chronic granulomatous disease (CGD). Genetic evaluation of the genes commonly associated with CGD (CYBB, CYBA, NCF1, NCF2 and NCF4) was negative. It must be noted that many genetic laboratories either do not sequence the NCF1 gene or have limited sequencing to the most commonly observed pathogenic variant in exon 2. Most targeted genetic panels currently do not include MPO (myeloperoxidase) or CYBC1 (Eros). The clinical phenotype of Candida infection in the context of diabetes has been previously reported in 5% of cMPO deficiency. The patient underwent a partial hepatectomy and was initiated on an 8-month course of fluconazole. Three months later, there was a recurrence of hepatic abscesses (Figure 1), requiring treatment with a 12-week course of IV micafungin. Complete MPO deficiency can cause a false-positive result on the DHR assay but the pattern is different from that observed in typical XL- and autosomal recessive CGD. There are two ways of confirming cMPO deficiency, besides genetic testing: 1) staining for MPO in neutrophils in a peripheral smear, or 2) repeating the DHR assay and gating on eosinophils as eosinophil peroxidase can overcome the lack of MPO deficiency and demonstrate a normal oxidative burst. These studies are currently under investigation. In summary, cMPO deficiency should be included in the differential diagnosis of patients with diabetes and invasive Candida infections. Communication with the clinical laboratory is essential to identify the DHR pattern and obtain additional confirmatory testing. From a clinical perspective, cMPO-deficient patients do not require prophylactic antibiotics but they have a high rate of recurrence of fungal infections and require aggressive management with antifungal therapies. [Display omitted]

Challenges of Diagnostic Approach in a Hyper-IgE Syndrome Family

Introduction: Hyper IgE syndrome (HIES), formerly known as Job’s syndrome, is an inborn error of immunity (IEI) with variable clinical presentations of infection, allergies, and high IgE levels. HIES has recently become better defined by genetic defects in STAT3, DOCK8, IL6R, and IL6ST, among others. Identifying defects in these genes can serve as a diagnostic method alongside scoring reports such as the NIH HIES scoring system. However, some patients may present with clinical manifestations that are unaccompanied by an identified pathogenic variant. Here, we highlight challenges in the diagnostic approach of HIES in an affected family. MethodsA retrospective data collection of the clinical history, symptoms, demographics, and treatment outcomes of the proband and his affected father was performed. Extensive immune subset phenotyping and functional assays on the signaling pathways were also conducted. Genetic evaluation was also completed and included 207-gene panel testing and whole exome sequencing. ResultsThe 15-year-old proband’s clinical history included significantly elevated IgE levels, eczema, lymphadenopathy, and severe, treatmentresistant herpes infections that led to two hospitalizations within the last year. His NIH HIES score was determined to be 45. His father experienced frequent herpes infections and recurrent abscesses and is also in remission for non-Hodgkin’s lymphoma. A 207 Invitae testing panel showed that neither individual had a pathogenic variant observed in STAT3 or DOCK8. Flow cytometric analysis revealed reduced memory and expanded CD19hi CD21lo B-cell populations in both the proband and father. Th2 skewing was seen in the CD4+ memory T cells of both individuals, along with increased fractions of CD4+ recent thymic emigrants and exhausted T cells. Notably, functional assays also revealed elevated phosphorylation of ERK, JNK, and AKT, while the phosphorylation of STAT5 in the naive compartment was lowered. Although these findings are not normally observed in STAT3 loss of function, they may be indicative of IL-2 signaling defects. ConclusionsDespite both the proband and the father exhibiting many clinical manifestations of HIES and scoring highly in the NIH HIES scoring system, the underlying IEI remains unidentified. Functional studies and extensive genetic analysis are vital for making clinical decisions and determining targeted therapies.

Epstein Barr Virus Positive Hodgkin's Lymphoma in a Patient with NEMO Deficiency

IntroductionHypomorphic mutations in the NF-kappaB essential modulator (NEMO) can manifest in diverse ways, but are best characterized by early susceptibility to viral, pyogenic and mycobacterial infections, ectodermal dysplasia and absent pneumococcal responses. We describe a case of EBV+ Hodgkin's lymphoma in a 9-year-old patient with NEMO on antimicrobial prophylaxis and immunoglobulin replacement therapy (IgRT). Case reportOur patient was diagnosed with NEMO deficiency at 2 years with initial presentation of recurrent pneumonia, recurrent skin abscesses and severe eczema herpeticum. His disease was moderately controlled with IgRT, and prophylaxis with trimethoprim/sulfamethoxazole, azithromycin and acylovir prophylaxis, with primary complications of continued recurrent pneumonia and finger clubbing. At 9 years of age however, he developed acute back pain and weakness, along with night sweats. MRI revealed thoracic spinal cord compression at T5 secondary to a possible neoplastic soft tissue mass. A laminectomy revealed Epstein Barr virus (EBV) positive Hodgkin lymphoma, stage 4 high risk due to PET positivity with uptake at large cervical chain nodal conglomerates bilaterally, mediastinal mass, thoracic paraspinal mass, periportal lymph nodes, splenic, and right medial femoral neck. He also had low level EBV viremia (EBV viral load 7,515 IU/mL). He was treated according to AHOD1331 with ABVE-PC (doxorubicin/bleomycin/vincristine/etoposide/prednisone), and also received rituximab and valganciclovir for treatment of EBV. He is now in remission after 4 months of treatment, and continues on IgRT, pneumocystis jiroveci pneumonia, mycobacterium avium complex, and herpes simplex virus prophylaxis. ConclusionPatients with certain inborn errors of immunity have a higher incidence of malignancy due to impaired immune function. Despite their viral susceptibility, EBV-induced lymphomas are not common in NEMO deficiency. NEMO patients may need further enhanced screening for development of malignancy, including regular surveillance for chronic, cancer-causing infections such as EBV and HPV.

Unique ulcerative autoinflammatory disease mistaken for factitious disorder

Autoinflammatory diseases (AID) are disorders of inappropriate innate immune system activation. Twenty-four percent of patients with AID do not have a well-defined clinical picture or pathogenic mutation and are characterized as having undefined AID. Cutaneous ulcers have been described but not outside of distinct histologic and clinical disorders. We present a unique case of undefined adult-onset AID with ulcers mistaken for a factitious disorder.A 32 year-old female with borderline personality disorder, depression, and anxiety developed recurrent cutaneous MRSA abscesses and recurrent ulcers of the extremities and scalp associated with alopecia. The ulcers were minimally-responsive to IV antibiotics, isotretinoin, oral dapsone, intralesional triamcinolone, and adalimumab. Flares of the skin disease were associated with fevers and elevated inflammatory markers.Scalp ulcer biopsies revealed numerous pigmented hair casts with trichomalacia and perifollicular lymphocytic infiltrate. One hair follicle contained bacterial cocci associated with a perifollicular neutrophilic infiltrate. These findings along with the patient's psychiatric history led to a diagnosis of trichotillomania from multiple dermatology and psychiatry consultations. The patient denied that she was inducing the lesions and sought another psychiatric consultation, who felt this was not a factitious disease. She was referred to immunology for evaluation of a possible IEI. Laboratory evaluation revealed mild hypogammaglobulinemia with IgG of 663 (768–1632) mg/dL, normal antibody response to recall antigens, but absent response to neo-antigen (Salmonella typhi). Peripheral lymphocyte phenotyping was normal except for a relative decrease in the number of memory B-cells with CD27+IgD+% of 6.9 (13.4–21.4) and CD27+IgD-% of 2.5 (9.2–18.9). Neutrophil oxidative burst was normal. Whole exome sequencing did not identify variants in genes associated with her phenotype.The patient was partially responsive to IVIG but highly responsive to high-dose intravenous methylprednisolone. She was subsequently diagnosed with undefined AID based on Eurofever registry criteria matching her clinical course and had near complete resolution of skin lesions and alopecia with the addition of anakinra.Clinicians must be vigilant for atypical presentations of AID. Our patient had signs of undefined AID but had a delayed diagnosis due to her psychiatric disorders, to which her symptoms were attributed, and a previously undescribed presentation of AID.

JAGN1 mutation with distinct clinical features; two case reports and literature review

Jagunal homolog 1 (JAGN1) has been recognized as an essential protein in neutrophil function. The mutated JAGN1 is responsible for immunodeficiency related to innate and humoral defense mechanisms. This deficiency impairs neutrophil development and function, leading to recurrent infections and facial dysmorphism as phenotypic consequences of severe congenital neutropenia (SCN). We report two siblings having the reported JAGN1 mutation with different clinical manifestations. Recurrent abscess formation unresponsive to antibiotic therapy, a history of delayed umbilical separation, frequent bacterial or fungal infection, dysmorphic face, failure to thrive, and other coexisting organ abnormalities should prompt physicians to syndromic immunodeficiencies involving neutrophils. Genetic investigations to elucidate the responsible mutation is critical as clinical management varies. Once the diagnosis is confirmed, a multi-disciplinary team should perform further workups to investigate other coexisting malformations and neurodevelopmental evaluation.

Incorporating the principles of oncoplastic surgery to treat recurrent breast abscess - Case report

Incorporating the principles of oncoplastic surgery to treat recurrent breast abscess - Case report