Idiopathic Granulomatous Mastitis, Erythema Nodosum, and Polyarthritis.

Idiopathic granulomatous mastitis (IGM) is an inflammatory-mediated rare disease that can be linked to rare manifestations. Erythema nodosum (EN) and polyarthritis, seen in a multitude of autoinflammatory and autoimmune diseases, have been rarely linked to IGM. Despite the cause of IGM being unclear, Corynebacterium infections are thought to play a role in the pathophysiology of IGM. Unusually, IGM has a relapsing and remitting course, which also applies to its systemic manifestations. As such, we present a case of IGM in a middle-aged lady who was initially thought to have Corynebacterium-containing unilateral abscesses for which drainage was performed. However, several abscesses devoid of bacterial growth started recurring, and the disease course was complicated by EN and polyarthritis. IGM, EN, and polyarthritis eventually resolved and were managed with symptomatic treatment.

Sarcoidosis as a cause of chronic back pain: a case report.

While sarcoidosis is typically a multisystem disease, it can, in some instances, exclusively affect the vertebrae, leading to back pain. Additionally, sarcoidosis may manifest with inflammation of the sacroiliac joints, not meeting the criteria for spondyloarthritis, yet contributing to back pain. In this report, we present a case involving a previously healthy 55-year-old woman who sought medical attention due to chronic back pain. She was subsequently diagnosed with spinal sarcoidosis, based on MRI, PET scan, and biopsy results. Furthermore, treatment with prednisolone monotherapy demonstrated substantial improvement in her symptoms.

Refractory Adult-onset Still's Disease Treated with a Combination of Methotrexate and Etanercept.

BACKGROUND: Adult-onset Still's disease (AOSD) is a challenging diagnosis because of the variability in clinical presentation and lack of gold-standard diagnostic investigations. Even after diagnosis, the treatment is challenging, especially when the disease is refractory to first-line therapy. Multiple pharmacotherapeutic options exist for refractory AOSD, but treatment failures still occur. Etanercept, a Tumor necrosis factor (TNF)-alpha inhibitor, is one of the options that has been rarely used for refractory AOSD, with various outcomes ranging from no response to complete remission. CASE PRESENTATION: In this case, we highlight how a previously healthy lady had refractory AOSD to glucocorticoids, methotrexate, and hydroxychloroquine combination therapy. There was no response to interleukin (IL)-1 therapy, which necessitated a switch to a combination of etanercept, low-dose methotrexate, and low-dose glucocorticoids with complete remission for a total of three- -year follow-up. CONCLUSION: The combination of methotrexate and Etanercept can maintain remission in patients with refractory AOSD.

Vacuolar Interface Dermatitis as a Histologic Reaction Pattern of Sjögren's Syndrome: A Case Report.

Sjögren's syndrome (SS) has been widely known for its dry mouth and dry eyes presentation. Extraglandular disease manifestations may be protean and pose a challenge for clinicians, especially when the typical known manifestations are absent. Skin involvement of SS is variable, and cutaneous signs and symptoms may be the initial presentation of this syndrome. Vacuolar interface dermatitis has been linked to dermatomyositis and systemic lupus erythematosus, but rarely to SS. Herein, we present the case of an 87-year-old man who presented for widespread itchy erythematous scaly plaques that were refractory to topical corticosteroids as well as discontinuation of possible offending medications. A biopsy demonstrated vacuolar interface dermatitis in the setting of strongly positive anti-SSA. Hydroxychloroquine treatment was effective in resolving the plaques.

Sarcoidosis Mimicking a Malignant Metastatic Disease.

Sarcoidosis can have pulmonary and extrapulmonary clinical manifestations depending on the organ of involvement. Because multiple organs are involved by the disease, sarcoid can mimic metastatic diseases. Whenever clinical and radiological clues of metastasis are present, differentials other than cancer should not be missed. Herein, we present a case of a middle aged gentleman who presented to the oncology clinic for 1-month history of low back pain associated with a dry cough along with radiological findings of metastatic disease involving the lungs, liver, lymph nodes, axial spine, and adrenal gland. A biopsy of the liver lesion showed non-caseating granuloma. Elevated blood levels of angiotensin-converting enzyme confirmed the diagnosis of sarcoidosis.

Clinical burden of hemophilia in older adults: Beyond bleeding risk.

The prospect of hemophilia patients has dramatically improved in the last few decades with the introduction of various interventions that can effectively treat or prevent their bleeding risk. The life expectancy of patients can now reach that of the healthy population, but this has paved the way for several previously unrecognized morbidities to manifest in older adults with hemophilia. Such clinical complications are attributed to suboptimal management or poor access to effective therapy during childhood as well as chronicity and prolonged exposure to the underlying pathophysiology of the disease and its treatment. Complications common in the aging population are also becoming increasingly relevant in this vulnerable patient subgroup. In this review, we highlight peculiarities of such morbidities including chronic viral infections and liver disease, debilitating joint impairment and bone disease, cardiovascular and chronic kidney disease, and cancers. We also reflect on topics of special interest in adulthood such as sexuality.

Complex vesicocutaneous fistula: Successful conservative management.

Vesicocutaneous fistulas are rare entities that could be either congenital or acquired. The diagnosis is usually based on clinical findings and imaging modalities. While most vesicocutaneous fistulas heal spontaneously, it is important to decrease the intravesicular pressure by diverting the urine. Moreover, surgical options are present to remove the fistula. In this case report, we highlight the case of a 67-year-old male, with recurrent obstructive cystitis and colorectal adenocarcinoma who developed a vesicocutaneous fistula. Decompression of the bladder led to complete closure of the tract.

Polymyalgia rheumatica-like presentation in a case of diffuse large B-cell lymphoma: a diagnostic pitfall.

Diffuse large B-cell lymphoma (DLBCL) commonly presents with systemic manifestations including fever, weight loss, and night sweats. Uncommonly, patients with DLBCL can present with musculoskeletal manifestations mimicking polymyalgia rheumatica (PMR). Herein, the case of a 61-year-old woman who presented with pain in the bilateral shoulders, arms, hands, knees, pelvic girdle, and neck with bouts of fever, is presented. Laboratory workup for infectious and connective tissue diseases was non-revealing, except for elevated inflammatory markers. A positron emission tomography (PET)/computed tomography (CT) scan was suggestive of PMR, but also revealed enlarged lymph nodes initially thought to be reactive in nature. However, a lymph node biopsy showed findings consistent with DLBCL. This case highlights the importance of a thorough investigational workup when cases with features of PMR do not meet the proper criteria for this diagnosis to be made, in order not to miss a hematopoietic neoplasm with a PMR-like presentation.

Characterizing the presence of neutrophil extracellular traps in neutrophilic dermatoses.

Neutrophil extracellular traps (NETs) are implicated in the pathogenesis of multiple inflammatory dermatoses. However, characterization of NETs in neutrophilic dermatoses was performed on very limited number of patients; this limitation precluded definitive conclusions. In this case series of 57 patients, we compared the amounts of neutrophils producing NETs in cutaneous lesions of different entities of neutrophilic dermatoses (17 with pyoderma gangrenosum, 37 with Sweet's syndrome and three with subcorneal pustular dermatosis). NETs were identified by double immunofluorescence on formalin-fixed paraffin-embedded skin biopsies using antibodies against elastase and citrullinated histone 3. Percentages of neutrophils showing NETs were high across all three entities (62.9% in PG, 48.5% in SS and 37.8% in subcorneal pustular dermatosis). The differences in mean percentages were significant between entities, with PG showing significantly superior percentage of NETs compared with SS. In our series, 15.8% of neutrophilic dermatoses were associated with malignancies, 10.5% with autoimmune diseases and 73.7% were idiopathic. Percentages of NETs were not statistically different between aetiologies. These findings suggest that NETs are abundantly produced in the various entities and different aetiologies of neutrophilic dermatoses. In comparison with SS, the superior percentage of NETs in PG is clinically mirrored in its greater ulceronecrotic nature.

Trans-urethral resection of bladder tumor (TURBT) and radiofrequency ablation of renal tumor: Rare etiologies of chyluria.

Chyluria is a rare entity which is caused by abnormal communication between the lymphatic system and the urinary tract, which allows the passage of chyle into the urine. Whenever symptomatic, chyluria presents with lower urinary tract symptoms such as dysuria and hematuria. Multiple imaging modalities can aid in diagnosis such as lymphoscintigraphy, retrograde pyelography, CT scan of the abdomen and pelvis, and magnetic resonance imaging. Numerous etiologies are associated with chyluria among which are surgical interventions. Partial and radical nephrectomy have been reported in a number of symptomatic and asymptomatic cases, while radiofrequency ablation of renal tumors has been very rarely reported. Herein, we report 2 cases of chyluria; one caused by trans-urethral resection of a bladder tumor and another by radiofrequency ablation of a renal oncocytoma. Fat-fluid level in the urinary bladder was seen on both precontrast and postcontrast images of CT scan of the abdomen and pelvis. Cystoscopy demonstrated no evidence of any abnormality of the urinary bladder.

Spontaneous rupture of the renal calyx secondary to a vesicoureteral junction calculus.

BACKGROUND: Spontaneous urinary collecting system rupture is caused by increased ureteral intraluminal pressure secondary to an obstruction. Rarely, a small stone exerts high intraureteral pressure especially if it is located distally. Many management modalities with good outcomes have been implicated. PURPOSE: Herein, we present a case of nontraumatic rupture of the renal calyx due to a 4 mm obstructing stone at the vesicoureteral junction. BASIC PROCEDURES: CT scan of abdomen and pelvis without contrast, CT scan of the abdomen and pelvis with intravenous contrast, Cystoscopy, Double-J ureter stent, Urinary Foley catheter. MAIN FINDINGS: The diagnosis was confirmed by CT imaging. Non-contrast enhanced CT scan of abdomen and pelvis showed obstructive calculi measuring 4 mm in the right vesicoureteral junction. Contrast-enhanced CT scan revealed leakage of contrast in the perinephric space at the right major calyx with intact bilateral ureters, suggestive of calyceal rupture. The treatment involved antibiotics and double-J stenting. PRINCIPAL CONCLUSIONS: This case demonstrates that spontaneous calyceal rupture should be suspected in urolothiasis patients presenting for a severe pain even if the calculus is small (less than 5 mm) and the laboratory markers are normal. An immediate management is required to relief symptoms and prevent further complications.

Intraparenchymal hemorrhage and cerebral venous thrombosis in an adult with congenital porencephalic cyst presenting for generalized tonic-clonic seizures.

Prothrombotic conditions are known risk factors for porencephalic cyst formation and cerebral vein thrombosis. Intracerebral hemorrhage is a potential complication of a cerebral vein thrombosis. Porencephaly is a risk factor for intracerebral hemorrhage and cerebral vein thrombosis formation. We present the case of an adult patient with a past medical history of epilepsy and congenital porencephalic cyst with de novo mutation of the COL4A1 gene who presented for episodes of generalized tonic-clonic seizure after a substantial symptom-free period. A brain CT scan showed an intracerebral hemorrhage with porencephalic cyst and superior sagittal sinus thrombosis despite negative thrombophilia work-up. A CT perfusion study, CT angiography, and brain MRI confirmed the diagnosis. The cause-and-effect relationship between porencephalic cysts, cerebral venous thrombosis, and intracerebral hemorrhage is still not clear in the literature.

Observational analysis of mesh related complications in urogynecologic procedures.

Since the 1990s, the use of mesh has expanded in gynecologic surgeries in the aim of correcting pelvic organ prolapse. Because there has been a lot of complications that were reported, the U.S. Food and Drug Administration has requested the withdrawal of many products from the market. Recently, many countries have called for the suspension of vaginal mesh products from the market. The objective of this review is to analyze the incidence of the complications that arise after vaginal mesh surgeries with an insight to the American College of Obstetricians and Gynecologists recommendations on the management of those complications.

Madelung's Disease Leading to Presenile Dementia in a Non-alcoholic Patient.

INTRODUCTION: Madelung's disease (MD) is a rare disorder of unknown etiology defined as the presence of multiple and symmetrical fatty accumulations most commonly involving the upper trunk, neck, and head. Excessive alcohol ingestion has been linked traditionally to the pathogenesis of the disease. The central and peripheral nervous system could both be affected. Presenile dementia, without alcohol abuse, has been rarely reported in the literature as a complication. AIM: The aim of this case report is to highlight that multiple symmetric lipomatosis can be complicated by presenile dementia even if the patient is non-alcoholic. CASE REPORT: This case report describes a middle age non-alcoholic woman who presented for increased forgetfulness. Brain CT scan showed cerebral and cerebellar atrophy inappropriate for her age. Despite being started on anticholinergic drug, her MMSE decreased 3 points in 1 year. CONCLUSION: Clinicians should consider early onset dementia as a potential complication of Madelung's disease even in patients with no preceding history of alcoholism. A brain MRI and MMSE can aid with identifying such a complication.

Update on the management of colchicine resistant Familial Mediterranean Fever (FMF).

BACKGROUND: Familial Mediterranean Fever (FMF), an autoinflammatory disease, is characterized by self-limited inflammatory attacks of fever and polyserositis along with high acute phase response. Although colchicine remains the mainstay in treatment, intolerance and resistance in a certain portion of patients have been posing a problem for physicians. MAIN BODY: Like many autoimmune and autoinflammatory diseases, many colchicine-resistant or intolerant FMF cases have been successfully treated with biologics. In addition, many studies have tested the efficacy of biologics in treating FMF manifestations. CONCLUSION: Since carriers of FMF show significantly elevated levels of serum TNF alpha, IL-1, and IL-6, FMF patients who failed colchicine were successfully treated with anti IL-1, anti IL-6, or TNF inhibitors drugs. It is best to use colchicine in combination with biologics.

Cerebral aspergillosis presenting as a space occupying lesion in an immunocompetent individual.

Cerebral aspergillosis has the tendency to occur in immunocompromised patients. Less commonly, immunocompetent individuals can be affected, with neuroimaging findings being difficult to interpret. The diagnosis necessitates imaging of the brain as well as the sinuses with biopsy and pathological confirmation. A surgical excision with aggressive antifungal agents are required for a proper management. This case report describes an immunocompetent patient with cerebral aspergillosis that presented radiologically as a suspicious mass to be diagnosed pathologically and excised surgically.

Uterine choriocarcinoma diagnosed 11 years after menopause: A case report.

BACKGROUND: Gestational trophoblastic neoplasms (GTNs) encompass a wide spectrum of diseases, of which choriocarcinoma is one of the most common. Choriocarcinoma occurs mainly in relation to pregnancy and rarely after the menopause. It has the potential to metastasize to organs other than the uterus. CASE REPORT: We describe a 62-year-old woman who presented with postmenopausal bleeding 11 years after the menopause. Pelvic ultrasound and abdominal/pelvic computerized tomography showed an intrauterine mass. Choriocarcinoma was diagnosed by Pipelle endometrial biopsy with positive staining for beta-human chorionic gonadotropin (hCG) and KI 67 along with an elevated serum beta-hCG level. The tumor was managed with multiple cycles of multidrug chemotherapy and follow-up based on serum beta-hCG levels according to the guidelines of the International Federation of Gynecology and Obstetrics (FIGO). CONCLUSION: This case report highlights that choriocarcinoma, a tumor normally associated with pregnancy, can present after the menopause.