An Unusual Case of Disseminated Erdheim-Chester Disease.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytic multisystem disorder, deriving from mononuclear phagocytic cells. It is notoriously challenging to diagnose. Here we present a case of a patient with multisystem ECD. CASE PRESENTATION: A 76-year-old female with a history of Hashimoto's thyroiditis who presented with persistent leukocytosis was found to have bilateral renal enlargement with a perinephric mass, a recurrent pericardial effusion, and bilateral pleural effusions. Following biopsies of several sites of involvement, a diagnosis of ECD was made. DISCUSSION: The existing literature on ECD is sparse, and no diagnostic criteria have been put forward due to widely differing presentations, although the most common is skeletal. Definitive diagnosis requires a tissue sample. CONCLUSIONS: In presenting our clinical reasoning and approach, we hope to contribute to the existing body of literature on ECD, with the aim of ultimately having sufficient data to compile a diagnostic framework for other clinicians who encounter ECD.

A Unique Case of Coexisting Anaplasmosis and Blastomycosis.

INTRODUCTION: In presenting this case of tick-borne illness in a patient with known disseminated blastomycosis, we aim to discuss the clinical reasoning and decision-making process when treating a septic presentation in a complex patient with multiple exposures and risk factors, from identifying and addressing the most devastating differentials to selecting appropriate empiric anti-infective regimens. CASE PRESENTATION: We present the case of a 60-year-old male with a medical history of diastolic heart failure, cirrhosis, sarcoidosis, hypertension, splenectomy, and recently diagnosed disseminated blastomycosis, who developed sepsis following a recent tick exposure. DISCUSSION: While a review of the literature revealed a paucity of cases of coexisting fungal and tick-borne illness, each is independently well-studied. Several reported commonalities exist between Blastomyces and Anaplasma, including endemic regions and at-risk populations.

Double Trouble: COVID-19 Pneumonia Concurrent With COVID-19-Associated Pulmonary Aspergillosis.

INTRODUCTION: Severe complications due to COVID-19 are a growing concern. We present a case of COVID-19 pneumonia with development of a superimposed COVID-19-associated pulmonary aspergillosis. CASE PRESENTATION: A 52-year-old unvaccinated male with a history of asthma and sleep apnea presented with progressive dyspnea 10 days after COVID-19 diagnosis. Worsening respiratory function despite broad-spectrum antibiotics and negative cultures prompted a repeat respiratory culture that revealed Aspergillus; voriconazole was initiated. DISCUSSION: The risk of COVID-19-associated pulmonary aspergillosis is highest in patients who are immunosuppressed or who receive corticosteroids to treat COVID-19 infection. Subtle and atypical presentations can be seen; our patient had only mild leukocytosis and progressive dyspnea with a negative initial respiratory culture. COVID-19-associated pulmonary aspergillosis is associated with high morbidity and mortality; thus, prompt diagnosis and treatment may confer a survival benefit. CONCLUSIONS: Despite the subtle presentation and variable radiographic findings in COVID-19- associated pulmonary aspergillosis, a low clinical threshold for workup is crucial to a timely diagnosis and treatment.

Listerial Spontaneous Bacterial Peritonitis.

Spontaneous bacterial peritonitis (SBP) is a severe complication of ascites often seen in advanced hepatic disease that is most commonly caused by Gram-negative bacilli. Here, we report a rare case of Listeria monocytogenes SBP, diagnosed by peritoneal fluid culture and responsive to ampicillin, in a patient with portal hypertension secondary to nodular regenerative hyperplasia. Because Listeria species are resistant to empiric SBP therapies and delays in treatment have been associated with increased mortality, they must be considered in high-risk patients.

Periclitoral Abscess: A Recurrent Problem.

BACKGROUND: Periclitoral abscesses are rare and lack a defined management strategy due to the limited number of cases available for review. Regardless of the initial management, recurrence remains common. CASE: A 17-year-old presented with clitoral pain and swelling consistent with periclitoral abscess. Conservative treatment with antibiotics failed, and underwent 2 incision and drainage procedures prior to resolution of the abscess. SUMMARY AND CONCLUSION: Spontaneous periclitoral abscess is rare, and the optimal treatment strategy has not been defined, with a high rate of recurrence being noted regardless of either conservative management with antibiotics or incision and drainage as initial treatment. In many cases of recurrent periclitoral abscess, hair was removed from the abscess cavity, suggesting that retained foreign material in the abscess cavity could be the nidus for recurrence.

Protein-Losing Enteropathy in Crohn's Disease: Two Unusual Cases.

Protein-losing enteropathy (PLE) occurs when protein losses throughout the gastrointestinal (GI) tract exceed the liver's ability to produce new protein. This is a rare presentation of Crohn's disease and few reported cases of PLE related to Crohn's exist in the literature. We describe two patients who presented with symptoms of PLE. After extensive diagnostic workup both were diagnosed with Crohn's disease and managed with prednisone.

Leave It to the Prose: A Case of POEMS Syndrome.

POEMS syndrome is a rare paraneoplastic syndrome named for its constellation of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. As a proliferative plasma cell disorder, it has a multisystem presentation and chronic cytokine overproduction. It often presents in the fifth or sixth decade of life, mostly in males. Presentations vary widely, making it extremely difficult to diagnose. A chronic progressive distal, sensorimotor polyneuropathy in conjunction with a monoclonal plasma cell dyscrasia suggests POEMS. A sixty-one-year-old man with a significant past medical history of bilateral lower extremity weakness and a cerebral meningioma presented with anorexia, intermittent dysphagia, and early satiety with intermittent vomiting and diarrhea over the past three months with a 20-pound weight loss. A CT of the abdomen and pelvis was obtained due to vomiting, showing expansile lesions of the left ischium, acetabulum, and ilium, with small-volume ascites and splenomegaly. Because of these findings and the noted bilateral lower extremity neuropathic weakness, we obtained further testing to corroborate suspicions of a plasma cell disorder. Labs were significant for IgG lambda with a monoclonal spike (M spike), low cortisol, low testosterone, and elevated thyroid-stimulating hormone (TSH). Protein electrophoresis was positive for bi-clonal lambda, and his vascular endothelial growth factor (VEGF) levels were elevated to 377. Bone marrow core biopsy indicated malignancy. Given his polyneuropathy, organomegaly, endocrinopathy, IgG lambda, skin changes, and extravascular fluid overload, POEMS syndrome was diagnosed. He was initiated on lenalidomide and dexamethasone and eventually received a stem cell autologous bone marrow transplant. Given his non-specific presentation, and the confounding factor of a known meningioma causing hypopituitarism, diagnosing POEMS required extensive workup. A contrast CT demonstrating bone lesions associated with myeloma-type disease was crucial, pairing his endocrinopathy and neuropathy with a plasma cell dyscrasia. Although POEMS is exceedingly rare, accurate diagnosis is vital, as treatment requires a multidisciplinary approach. While high-dose chemotherapy-conditioned autologous stem cell transplantation is the gold-standard treatment for POEMS syndrome, patients who are diagnosed in a late stage of the disease are not candidates, underlining the need for early identification of the disorder.

Point-of-care Doppler ultrasound in the management of hyponatremia: Another string to nephrologists' Bow.

Objective assessment of fluid status is vital for the appropriate management of patients with hyponatremia. Conventional physical examination suffers from several limitations in this regard, and point-of-care Doppler ultrasonography can be used as an adjunct to clinical and laboratory data in evaluating these patients.