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Uncommon Mimics the Rare: Lipid Versus Paraproteinemic Keratopathy in a Patient With Smoldering Multiple Myeloma | Annals of Internal Medicine: Clinical Cases

A 69-year-old man with smoldering IgA lambda multiple myeloma developed progressive bilateral peripheral corneal opacities initially concerning for paraproteinemic keratopathy (PK). Despite recommendations for systemic therapy, histopathologic analysis of corneal tissue obtained during penetrating keratoplasty (PKP) demonstrated lipid-laden stromal deposits without immunoglobulin staining, confirming lipid keratopathy (LK). This case illustrates the diagnostic challenge of distinguishing LK from PK in patients with monoclonal gammopathies and emphasizes the need for histopathologic confirmation before initiating systemic therapy. Accurate differentiation can prevent unnecessary treatment and guide appropriate management of corneal deposition disorders associated with plasma cell dyscrasias.

Cryptococcal Meningoencephalitis in an HIV-Negative Host Infected With COVID-19: A Case Report | Annals of Internal Medicine: Clinical Cases

We report a rare case of cryptococcal infection in an immunocompetent adult who initially presented with altered sensorium and acute COVID-19 infection. The suspected diagnosis was encephalopathy due to pneumonia; however, the patient developed new neurologic deficits, warranting further investigation, which revealed cryptococcal meningoencephalitis. HIV testing was negative, but CD4 lymphocytopenia was seen, which resolved after treatment for both COVID-19 and cryptococcal meningoencephalitis. This case illustrates the importance of considering uncommon scenarios in which an opportunistic infection such as cryptococcal meningoencephalitis may present. It also demonstrates the need to frequently consider alternative diagnoses, particularly in the pandemic era.

Recurrent Cryptogenic Stroke in a Young Woman: Congenital Thrombotic Thrombocytopenic Purpura Unmasked | Annals of Internal Medicine: Clinical Cases

Cryptogenic stroke of undetermined cause should warrant an exhaustive neurologic and cardiovascular workup. If no cause is identified, additional investigations should be individualized on the basis of clinical history and objective findings. Herein, we present the case of a young patient with a personal and family history of cryptogenic stroke who was investigated for thrombophilia. Transient thrombocytopenia and peripheral blood schistocytes led to an eventual diagnosis of congenital thrombotic thrombocytopenic purpura, which is characterized by vaso-occlusive end-organ complications. The diagnosis is confirmed by undetectable ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs, member 13) activity.

Dermatitis Neglecta: A Case Report of Hyperpigmentation Resulting From Impaired Mechanical Cleansing | Annals of Internal Medicine: Clinical Cases

Dermatitis neglecta (DN) results from inadequate frictional cleansing and is often associated with psychiatric or neurologic impairments. It is frequently misdiagnosed owing to its rarity and variable presentation. We report a 71-year-old woman with extensive hyperpigmented plaques from the mid-back to posterior thighs. Initially suspected to be tinea corporis, the primary lesion did not fluoresce on Wood's lamp examination. Mechanical cleansing with soap, water, and alcohol swabbing removed the pigmentation, confirming DN. This case emphasizes the importance of recognizing DN, particularly in patients with functional limitations, to avoid unnecessary testing and ensure effective management.

An Unusual Case of Hyperosmolar Hyperglycemic State Complicated by Bilateral Occipital Lobe Seizures | Annals of Internal Medicine: Clinical Cases

Hyperosmolar hyperglycemic state (HHS) is a complication of type 2 diabetes mellitus (T2DM) marked by hyperglycemia without acidosis. We present a case of a 59-year-old woman with a history of T2DM and migraines who presented with worsening occipital headaches accompanied by visual disturbances. Her glucose level on arrival was 559 mg/dL with a normal anion gap. Magnetic resonance imaging revealed cortical restricted diffusion in the left occipital lobe. An electroencephalogram detected electroclinical focal seizures in the bi-posterior region. This case highlights the importance of both recognizing seizures in HHS and the need to consider alternative diabetes diagnoses for patients with atypical diabetic phenotypes.

Beyond Uremia: Chronic Kidney Disease as a Cryptic Gateway for Opportunistic Neuroinvasion by John Cunningham Virus | Annals of Internal Medicine: Clinical Cases

Progressive multifocal leukoencephalopathy (PML), a devastating demyelinating central nervous system infection caused by John Cunningham virus (JCV) reactivation, typically occurs in profound immunosuppression. Chronic kidney disease (CKD) induces a state of “immunoparalysis” through uremic toxin-mediated T-cell dysfunction yet remains an underrecognized risk factor for PML. We present a 72-year-old man with end-stage renal disease who developed subacute expressive aphasia and confusion. Magnetic resonance imaging showed demyelinating white matter lesions, and cerebrospinal fluid confirmed JCV DNA, establishing PML. This case highlights CKD as a cryptic immunosuppressive state predisposing to PML and emphasizes the importance of considering PML in patients with CKD presenting with new, progressive neurologic deficits.

Leptomeningeal Immunoglobulin G4–Related Disease: A Case Report | Annals of Internal Medicine: Clinical Cases

We present the case of an older man with history of rheumatoid arthritis, taking etanercept who developed focal neurologic deficits with imaging findings that raised concern for central nervous system neoplasia, as well as a nondiagnostic initial brain biopsy. Imaging showed left hemispheric leptomeningeal enhancement, as well as left frontal convexity rim-enhancing collections, suspicious for abscess. Empiric antibiotics did not improve the patient's condition, and a second central nervous system biopsy revealed a diagnosis of leptomeningeal immunoglobulin G4–related disease, a rare fibroinflammatory condition. Awareness of this condition, prompt diagnosis, and treatment are imperative to prevent irreversible organ damage, disability, and potentially death.

Posterior Reversible Encephalopathy Syndrome in an Older Patient in the COVID-19 and Paxlovid Era | Annals of Internal Medicine: Clinical Cases

Nirmatrelvir/ritonavir (Paxlovid) has been prescribed increasingly for treatment of COVID-19. Due to ritonavir's inhibition of CYP3A4, there are many medication interactions to consider. In this case, a 63-year-old woman recently treated with Paxlovid presented with symptoms mimicking previous episodes of lupus cerebritis. Imaging, however, revealed a pattern more consistent with posterior reversible encephalopathy syndrome. When Paxlovid therapy was initiated in the ambulatory setting, her amlodipine dose was decreased due to concern for interactions. Since Paxlovid is recommended for patients with high risk for progression to severe illness, this poses a unique challenge, especially in older patients with multiple comorbid conditions.

Cerebral Fat Embolism in a Patient With Hemoglobin SC Disease Associated With Vaso-Occlusive Crisis and COVID-19 | Annals of Internal Medicine: Clinical Cases

Sickle cell disease is characterized by episodic vaso-occlusive events triggered by a combination of inflammation, increased adhesion to vascular endothelial cells, and activation of hemostasis. This vascular obstruction results in acute vaso-occlusive pain, stroke, and acute chest syndrome, which are more commonly observed when patients are admitted for acute vaso-occlusive crisis. Although fat embolism syndrome is most associated with orthopedic trauma, individuals with sickle cell hemoglobinopathy are at increased risk for nontrauma-related fat embolism syndrome. Here we describe a young woman with a history of hemoglobin SC disease who presented with fat embolism syndrome following a vaso-occlusive crisis precipitated by COVID-19 infection.

α-Gal Syndrome: Busting Paradigms in Food Allergy | Annals of Internal Medicine: Clinical Cases

α-Gal syndrome, also known as red or mammalian meat allergy, results from immunoglobulin E–mediated hypersensitivity responses to the carbohydrate galactose-α-1,3-galactose (α-gal). Patients with α-gal syndrome experience immediate onset of allergic symptoms following the injection of pharmaceutical products containing α-gal. However, it typically takes 2 hours or more after dietary α-gal ingestion before patients with α-gal syndrome experience immunoglobulin E–mediated hypersensitivity responses. The case report by Heffes-Doon and colleagues highlights the lack of official guidelines on when and how to reintroduce mammalian meat products into the diet when there is clear laboratory evidence of declining α-gal immunoglobulin E levels.

Sorry, no results were found for "im_matters_articles" in ACP Gastroenterology Monthly.