Elusive Entity Research Articles

Mechanisms of benefits of sodium-glucose cotransporter 2 inhibitors in heart failure with preserved ejection fraction.

For decades, heart failure with preserved ejection fraction (HFpEF) proved an elusive entity to treat. Sodium-glucose cotransporter 2 (SGLT2) inhibitors have recently been shown to reduce the composite of heart failure hospitalization or cardiovascular death in patients with HFpEF in the landmark DELIVER and EMPEROR-Preserved trials. While improvements in blood sugar, blood pressure, and attenuation of kidney disease progression all may play some role, preclinical and translational research have identified additional mechanisms of these agents. The SGLT2 inhibitors have intriguingly been shown to induce a nutrient-deprivation and hypoxic-like transcriptional paradigm, with increased ketosis, erythropoietin, and autophagic flux in addition to altering iron homeostasis, which may contribute to improved cardiac energetics and function. These agents also reduce epicardial adipose tissue and alter adipokine signalling, which may play a role in the reductions in inflammation and oxidative stress observed with SGLT2 inhibition. Emerging evidence also indicates that these drugs impact cardiomyocyte ionic homeostasis although whether this is through indirect mechanisms or via direct, off-target effects on other ion channels has yet to be clearly characterized. Finally, SGLT2 inhibitors have been shown to reduce myofilament stiffness as well as extracellular matrix remodelling/fibrosis in the heart, improving diastolic function. The SGLT2 inhibitors have established themselves as robust, disease-modifying therapies and as recent trial results are incorporated into clinical guidelines, will likely become foundational in the therapy of HFpEF.

Surgical Unroofing of Myocardial Bridges

•Surgical unroofing via standard median sternotomy or minimally invasive approaches stands as an effective treatment for symptomatic and hemodynamically significant myocardial bridges. Myocardial bridges (MB) are a congenital variant in which an epicardial coronary artery—most commonly the left anterior descending (LAD)—is contained within an intramyocardial segment. The clinical significance of this lesion varies from no symptoms to debilitating angina, ventricular arrhythmia, and even sudden cardiac arrest. Traditionally difficult to diagnose, symptomatic myocardial bridges remain an elusive entity with a lack of clarity regarding medical treatment and surgical indications. Importantly, studies have shown that with appropriate patient selection, MB unroofing is an effective treatment for symptomatic lesions, in both the adult and pediatric populations. This article describes the preoperative evaluation and the surgical approach for myocardial bridges. Myocardial bridges (MB) are a congenital variant in which an epicardial coronary artery—most commonly the left anterior descending (LAD)—is contained within an intramyocardial segment. The clinical significance of this lesion varies from no symptoms to debilitating angina, ventricular arrhythmia, and even sudden cardiac arrest Traditionally difficult to diagnose, symptomatic myocardial bridges remain an elusive entity with a lack of clarity regarding medical treatment and surgical indications. Importantly, studies have shown that with appropriate patient selection, MB unroofing is an effective treatment for symptomatic lesions, in both the adult and pediatric populations. This article describes the pre-operative evaluation and the surgical approach for myocardial bridges. Myocardial bridges (MB) are a congenital variant in which an epicardial coronary artery—most commonly the left anterior descending (LAD)—is contained within an intramyocardial segment. The clinical significance of this lesion varies from no symptoms to debilitating angina, ventricular arrhythmia, and even sudden cardiac arrest Traditionally difficult to diagnose, symptomatic myocardial bridges remain an elusive entity with a lack of clarity regarding medical treatment and surgical indications. Importantly, studies have shown that with appropriate patient selection, MB unroofing is an effective treatment for symptomatic lesions, in both the adult and pediatric populations. This article describes the pre-operative evaluation and the surgical approach for myocardial bridges.

Unmasking of a spinal dural AV fistula on MRI following steroid administration

BackgroundSpinal dural arteriovenous fistulas (dAVF) remain a rare, diagnostically challenging and possibly correctable condition with important prognostic outcomes dependent primarily on early detection and treatment.Case presentationWe present a case of a 73-year-old male with progressively worsening neurological symptoms after steroid administration for a presumed diagnosis of transverse myelitis. This case is extremely unique, as the administration of steroids helped unmask an underlying spinal dural arteriovenous fistula by revealing characteristic imaging findings of a dAVF, not seen on the original MRI study.ConclusionIn the setting of an unclear cause of transverse myelitis and worsening symptoms following steroid administration, the possibility of a ‘masked’ dural AVF should be considered and repeat imaging performed, which might help in the eventual diagnosis and definitive treatment of this elusive entity.

Aslak Rostad. Human Transgression—Divine Retribution: A Study of Religious Transgressions and Punishments in Greek Cultic Regulation and Lydian-Phrygian Propitiatory Inscriptions (“Confession Inscriptions”)

Aslak Rostad. <i>Human Transgression—Divine Retribution: A Study of Religious Transgressions and Punishments in Greek Cultic Regulation and Lydian-Phrygian Propitiatory Inscriptions (“Confession Inscriptions”)</i>

Kohler’s disease mimicking osteomyelitis: A case report

Kohler’s disease is a rare cause of limping among pediatric population due to impaired blood supply to tarsal navicular bone. It is a condition of unknown etiology affecting children between 3-7 years of age with male preponderance. It is a good mimicker of other common causes of a limping child which is an elusive entity for physicians. Adequate clinical suspicion and knowledge is paramount for proper utilization of advanced diagnostic tools and to prevent a delayed diagnosis.Here we present a case of Kohler’s disease in a 5 years-old boy with clinical features mimicking osteomyelitis.

On the reliability and validity of central fatigue determination

Physical performance fatigue can be ascribed to both peripheral and central components. Central fatigue, however, is an elusive entity, consisting of cognitive/sensory component and presumably also a neuro-physiological component that are difficult to tease apart and assess independently of each other. The most widely accepted method for the assessment of central fatigue is based on the premise that decreasing volitional muscle activation (VA), as determined by the interpolated twitch technique (ITT) in fatiguing muscles, reflects increasing central fatigue. Suffering its own shortcomings, the validity of VA determination under fatigued conditions has never been proven and is only assumed. This review presents evidence that questions ITT's reliability and validity in reflecting VA in the fatiguing muscle and, consequently, VA's validity for central fatigue assessment. Specifically highlighted is the paradox of children and endurance athletes, who share striking endurance characteristics, being claimed as more centrally fatigable than untrained adults. Further research and new directions are needed for confirming and quantifying central fatigue and teasing apart its psychologic and neuromotor components.

Is heart failure with preserved ejection fraction a 'dementia' of the heart?

Heart failure with preserved ejection fraction (HFpEF) remains an elusive entity, due to its heterogeneous clinical profile and an arbitrarily defined nosology. Several pathophysiological mechanisms recognized as central for the development of HFpEF appear to be in common with the process of physiological aging of the heart. Both conditions are characterized by progressive impairment in cardiac function, accompanied by left ventricular hypertrophy, diastolic dysfunction, sarcomeric, and metabolic abnormalities. The neurological paradigm of dementia-intended as a progressive, multifactorial organ damage with decline of functional reserve, eventually leading to irreversible dysfunction-is well suited to represent HFpEF. In such perspective, certain phenotypes of HFpEF may be viewed as a maladaptive response to environmental modifiers, causing premature and pathological aging of the heart. We here propose that the 'HFpEF syndrome' may reflect the interplay of adverse structural remodelling and erosion of functional reserve, mirroring the processes leading to dementia in the brain. The resulting conceptual framework may help advance our understanding of HFpEF and unravel potential therapeutical targets.

Kleine-levin syndrome

IntroductionKleine-Levin Syndrome (KLS) is an extremely rare disorder of unknown etiology. It affects mainly male adolescents and is thought to follow a relapse-remitting course. During episodes of illness, a wide array of neuropsychiatric symptoms may present and a psychiatric diagnosis might be incorrectly made.ObjectivesWe aim to review the literature on the clinical manifestations of KLS, as well as the current evidence regarding this disorder’s management.MethodsWe performed an updated review in the PubMed database using the terms “Kleine-Levin Syndrome”. The included articles were selected by title and abstract.ResultsKLS usually presents with recurrent episodes, lasting days to weeks, of severe hypersomnia, cognitive impairment, major apathy and derealization, among other neuropsychiatric symptoms. Although it was previously thought that complete normalization occurred between episodes, recent evidence suggests that around one third of patients have mild cognitive impairment and there are alterations in brain blood flow during the asymptomatic periods. During episodes of illness, management comprises environmental measures as well as drug therapy. Corticosteroids and amantadine have been successful in stopping episodes and lithium may be useful in a preventative role, however, there are no randomized controlled trials focusing on KLS treatment.ConclusionsKLS remains an elusive entity since it is an extremely rare disorder with unclear etiology, course, and no consensual treatment. Further research is warranted in this area, namely randomized controlled trials. It is important for the practicing psychiatrist to be aware of this illness in order to recognize it and adequately manage it.

Role of T1 mapping in identification of convalescent Takotsubo cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Background Takotsubo cardiomyopathy remains an elusive entity to diagnose, especially with increasing time following the initial clinical event. Patients are often referred for cardiac MR, but when performed on a convalescent patient, the conventional CMR exam can be normal. However CMR elevated native T1 values on mapping may persist, aiding diagnosis. Purpose We sought to review a series of confirmed Takotsubo cases to evaluate which CMR features were most helpful in making a diagnosis. Methods We reviewed 2 years of CMR exams in our institution and identified 14 patients with a confirmed diagnosis of Takotsubo cardiomyopathy after a read of all clinical data by two consultant cardiologists. All of the 14 patients had a troponin positive cardiac event, unobstructed coronary arteries on invasive angiography and echocardiographic evidence of apical hypo/akinesia (or basal, in the non-apical variants) at the time of presentation. All CMR exams were performed at 1.5T. All included functional assessment, T1 mapping and late gadolinium enhancement(LGE). Some exams also included T2 mapping and STIR. Results All patients were female. The time interval between presentation and CMR exam varied from 24 hours to 2 years. 2 of the scans showed evidence of non-apical Takotsubo cardiomyopathy. At the time of CMR, only 7 patients(50%) had a residual regional wall motion abnormality, but 13 patients(93%) had a region of elevated native T1 (&amp;gt;1 myocardial segment). High signal was identified on STIR in 6(43%) and elevated native T2 in 10(71%). Of interest, we identified small areas of patchy LGE in 2 patients(14%). Conclusion Increased native T1 signal may be the only residual cardiac MRI marker of previous Takotsubo cardiomyopathy, persisting for months after the initial cardiac event, even after resolution of regional wall motion abnormalities and recovery of the left ventricular function. T1 mapping should be performed in CMR exams for Takotsubo cardiomyopathy to improve diagnostic yield. Abstract Figure. CMR in convalescent Takotsubo

Malignancy rates in thyroid nodules classified as Bethesda categories III and IV; a subcontinent perspective

BackgroundBethesda category III and IV thyroid nodules fall in the indeterminate risk of malignancy category. These nodules have been a relatively elusive entity to manage as previous studies have shown a wide variation in malignancy rates in different regions and institutions across the world. However, data from the subcontinent with regards to this is scarce. Aim and objectiveThis study aimed to determine the characteristics and malignancy rates of cytology proven Bethesda Category III and IV thyroid nodules and its association with clinical, histopathological and laboratory variables, in the regional population. MethodA retrospective search was performed on all patients with thyroid nodules who presented to this hospital, from January 2011 to September 2018. Patients who had cytology proven Bethesda category III and IV thyroid nodules that underwent surgery were included in the study. ResultsMalignancy in Bethesda Category III and Bethesda Category IV thyroid nodules was 29.6% and 47.1%, respectively. There was no significant association determined between malignancy rate and various clinical, histopathological, and radiological characteristics. ConclusionThe malignancy rates in Bethesda category III and IV thyroid nodules in this study are significantly higher than that initially suggested by the Bethesda consensus publication but is comparable to international data present.

Surprise, Event and the Metonymic Subject

A well-worn platitude holds that surprise (admiration for the Greeks, but closer to astonishment for their successors) is the starting point of philosophy (while carefully distinguishing philosophical surprise from the routine kind). Surprise also functions as an important concept in aesthetics. Russian formalism elucidates it through the concepts of ostranenie and defamiliarization. Roman Jakobson in 1919 began a heuristically rich analysis that reveals the role of metonymy in prose and new poetry — in contrast to the centrality of metaphor in traditional poetry. This reassessment of the role of contiguity (as well as of randomness and arbitrariness) as opposed to similarity (or, stated another way, syntax vs. paradigmatics) has found some resonance in (mainly, but not exclusively, French) thinking about the concept of event. After its introduction in Gilles Deleuze’s The Logic of Sense (1969), the real apotheosis of the event unfolds among different thinkers (Derrida, Badiou, neo-phenomenologists) who glorify the unexpectedness, unforeseeableness, ineffability and causelessness of the event. The article shows that this kind of event theorizing (with its inherent optimism and joyous enthusiasm for new horizons and possibilities) is bound to the type of event within which that thinking took shape, the “revolution” (as it conceived itself) of May 1968. This “theory” cannot fully account for either certain previous events (the Holocaust) nor subsequent ones (such as 9/11 or the pandemic). Parallel with the thinking about the May 1968 event came reflections on subjectivity, or rather on the rapid alteration in its historical types during the 20th century accompanied by efforts to grasp what has taken the place once occupied by the subject. This new and elusive entity (referred to as advenant or “coming about” by Claude Romano) corresponds to the metonymic, contingent nature of current social and/or semantic reality itself.

Reckoning the Unresolved Scientific Question on Memory Transfer

Memory formation occurs within the central nervous system (CNS), specifically in the hippocampal region of brain. The notion that memories are only located within the brain has been challenged by reports of some patients that they have “inherited memories” from their donor after organ transplantation; some even experienced personality changes and picked up hobbies or preferences similar to their donor. Recently, a research team has reignited the embers of this theory by using scientific method to show that memory can be genetically transferred from one sea snail to another. Nevertheless, even as more and more scientific mysteries are being unravelled, memory remains an elusive entity shrouded in the haze of many unresolved hypotheses. To seek clarity on what is currently known, this write-up summarizes and consolidates records associated with the theory of “cellular memory” and experiments evaluating the possibility of memory transference by genetic materials like RNA.

Small Bowel Transplant—Observations from Initial Setback

Small bowel transplants are performed all over the world with acceptable success rates. Although there are isolated reports of small bowel transplants from India, a robust intestinal transplant programme similar to western countries is lacking. Our aim is to share our experience to build up on the evolving field of small bowel transplants in India. Deceased donor small bowel transplant was performed for three patients with intestinal failure, two for short gut syndrome following surgery and one for visceral myopathy. All were on preoperative total parenteral nutrition for varying periods of time (6 to 13 months). There were multiple episodes of line sepsis in all patients. Post-transplant, all received induction immunosuppression with anti-thymocyte globulin (first two patients) or alemtuzumab (3rd patient). All had systemic venous drainage and stoma. Protocol weekly intestinal biopsies were done in all patients. All died in hospital at 24, 12 and 28 days following surgery. Biopsy-proven rejection was observed in only patient who had received ABO-compatible non-identical graft (O to A). This patient subsequently developed vascular thrombosis necessitating explant of the graft. Sepsis due to multidrug resistant bacteria was the reason for mortality in the remaining two patients. Despite the absence of technical complications, successful small bowel transplant seems to be an elusive entity for the Indian transplant community.

MON-701 Uncontrolled Diabetes Presenting as Isolated Sixth Nerve Palsy

BACKGROUND Our case report demonstrates acute onset of diplopia due to Isolated Sixth Nerve Palsy (ISNP) secondary to uncontrolled T2DM, presenting as an ophthalmoplegia. The most frequent one, is ISNP with an incidence of 11.3/100.000 1. Vasculopathic ISNP is associated with atherosclerosis in patients older than 50 years 1.CASE PRESENTATION A 63 year-old male with history of T2DM, HTN, HL, came for evaluation of acute onset double vision 3 days prior. He noted diplopia while attempting to park his car; he saw that tracking to the left with his eyes would elicit double vision. Denied recent travel, trauma, headache or dizziness. He was awake and alert, BP 200/110mmHg, BMI 33. No pathologic murmur. He had PERRLA bilaterally but impaired lateral rectus muscle movement on the left side. Otherwise, EOMI on the right side. No facial asymmetry or ptosis. Overall, findings positive for ISNP. BMP only remarkable for a glucose of 297, HA1c was 10.0. Head CT was negative for any acute intracranial abnormality. Orbital MRI did not show acute infarction or masses. Patient was admitted for acute diplopia due to ISNP. Differential diagnoses were neoplasm, migraine, MS and diabetic neuropathy. Based on the aforementioned data, we suggested that T2DM was the probable cause. Counseling on improving glycemic control was given. Unfortunately, patient was lost to follow up. DISCUSSION ISNP remains an elusive entity; atherosclerotic risk factors such as DM, HTN, HL, hyperhomocysteinemia 2 or viral infections 3 have been reported in association. This type of palsy seems to be more frequent in children and can be recurrent in nature.In adults, the most likely cause of ISNP seems to be ischemic mononeuropathy or more aggressive etiologies such as temporal arteritis 4. Inconclusive images prove even a higher diagnostic challenge 3. Of note, we found a case demonstrating evidence for Eicosapentaenoic Acid in the improvement of ISNP with recovery in as shortly as 8 weeks. The basis of this treatment lies in the recovery of endothelial function focusing on the anti-platelet and anti-inflammatory effects of the drug2,4. REFERENCES (1). Elder, Christopher, et al. “Isolated abducens nerve palsy: update on evaluation and diagnosis.” Current neurology and neuroscience reports 16.8 (2016): 69. (2). Takenouchi, Yasuhiro, et al. “Eicosapentaenoic acid ethyl ester improves endothelial dysfunction in type 2 diabetic mice.” Lipids in health and disease 17.1 (2018): 118. (3). Azarmina, Mohsen, and Hossein Azarmina. “The six syndromes of the sixth cranial nerve.” Journal of ophthalmic & vision research 8.2 (2013): 160. (4). Yanai, Hidekatsu, and Mariko Hakoshima. “Eicosapentaenoic Acid for Diabetic Abducens Nerve Palsy.” Journal of Endocrinology and Metabolism 7.4 (2017): 131–132.

Distinguishing atypical chronic myeloid leukemia from other Philadelphia-negative chronic myeloproliferative neoplasms.

Atypical chronic myeloid leukemia (aCML), BCR-ABL1-negative, is a rare myelodysplastic/myeloproliferative neoplasm (MDS/MPN) characterized by leukocytosis, granulocytic dysplasia, and typically poor patient outcomes. Since its first description as a variant CML lacking the Philadelphia chromosome (Ph), the diagnostic criteria for aCML have evolved significantly. Nevertheless, distinguishing it from other Ph-negative myeloid neoplasms can still be very challenging, and given its generally worse prognosis, this is a clinically important distinction. The purpose of this review is to conceptualize our understanding of aCML molecular genetics based on recent advances, and describe how genetic features can be used in conjunction with clinical and morphologic features to better diagnose this elusive entity. The classification criteria for aCML have evolved and changed multiple times over the past decades, and is now based on strict application of morphologic, clinical and laboratory criteria. Recent work has elucidated the mutational landscape of aCML, especially with respect to potentially differentiating profiles compared with other Ph-negative myeloid neoplasms. Atypical CML is a rare MDS/MPN overlap syndrome that can be diagnostically challenging; however, its emerging molecular genetic understanding and clinicomorphologic phenotype can help in distinguishing it from other Ph-negative myeloid neoplasms.

Chronic thromboembolic pulmonary hypertension in patients with persistent chest symptoms after acute pulmonary embolism.

This study aimed to analyze the role of chronic thromboembolic pulmonary hypertension (CTEPH) in patients with persistent chest symptoms after acute PE. Patients aged between 18 and 80 years who were followed up for acute PE were screened for chest symptoms which persisted after the anticoagulation treatment. Patients suffering other types of pulmonary hypertension (PH) or metastatic malignancies were excluded in this study. Demographic and functional data of patients included this study were collected. The patients underwent transthoracic echocardiography and ventilation/perfusion (VQ) scans. Also, invasive hemodynamic studies were done to patients with intermediate/high probability of VQ scans. Of the 140 patients screen for this study, 29 patients (Female/Male=16/13) with mean age of 56.1±11.2 years and follow-up time of 35.1±17.7 months met the inclusion criteria. The mean systolic pulmonary artery blood pressure (sPAP) on transthoracic echocardiography was 28.9±4.9 mm Hg (range=20-40 mm Hg). Furthermore, intermediate or high probability of VQ scans was detected in 2 patients, who were subsequently diagnosed with CTEPH (6.9%) via right heart catheterization. CTEPH was diagnosed at a low rate in patients with persistent chest symptoms after the anticoagulation treatment for PE. CTEPH is still an elusive entity, which requires a multidisciplinary and invasive approach.

Upper Gastrointestinal Crohn’s Disease: Literature Review and Case Presentation

Upper gastrointestinal tract predominant Crohn's Disease (CD) remains an elusive clinical entity, manifesting limited or vague symptomatology, eluding clinical suspicion, and delaying subsequent diagnostic evaluation. As a result, it has not been widely described and there is a lack of clear recommendations for diagnosis or management. Standard IBD evaluation including serologic testing, imaging, and endoscopy may initially not be fruitful. Furthermore, endoscopic evaluation may be grossly normal in patients without long standing-disease. We describe an 18-year-old male who presented with only unexplained, persistent iron-deficiency anemia. Extensive outpatient testing including multiple endoscopic evaluations with standard biopsies was unfruitful. Ultimately, a positive fecal calprotectin prompted enteroscopy with endoscopic mucosal resection (EMR) in an effort to obtain a larger, deeper tissue specimen. Grossly cobblestoned mucosa along with histopathology revealing focal crypt abscesses, chronic inflammation in the lamina propria, and superficial foveolar epithelial regenerative changes were consistent with CD. This patient's case illustrates the need for a high degree of suspicion for CD in patients with unexplained or persistent iron deficiency anemias. Persistent investigation yielded an elevation in fecal calprotectin suggesting underlying gastrointestinal inflammation and prompted advanced endoscopic evaluation with EMR. Waxing and waning tissue findings are characteristic of CD and pose a unique challenge in patients with upper gastrointestinal predominant pathology. As such, diligent workup including laboratory evaluation, imaging, and serial endoscopy is critical to establish pathology and dictate subsequent management in IBD, especially upper gastrointestinal tract predominant CD.

First TMEM126A missense mutation in an Italian proband with optic atrophy and deafness.

Recessively inherited optic neuropathy has been an elusive entity for a long time. Currently, a few causative genes have been described,1–6 associated with a spectrum of isolated or syndromic optic atrophy. Among these genes, TMEM126A (OPA7) was the first to be reported, with a single causative mutation found in all pedigrees identified to date of North African ancestry (c.163C>T; p.Arg55X), thus possibly belonging to the same founder mutational event.1,7,8

Music Video in its Contexts: 30 Years Later

Thirty years after I wrote the article reproduced elsewhere in this issue of Volume, the videoclip is an elusive entity. In the present moment it has become, it would seem, both ubiquitous and minor. Through the videoclip, YouTube has become the largest disseminator of music in the world, but this has diminished rather than magnified the clip’s status as cultural form. The abundance of clips on Youtube seems a sign of little more than that platform’s uncontrolled voraciousness and role as dum...

AL Amyloidoma of the Skin/Subcutis: Cutaneous Amyloidosis, Plasma Cell Dyscrasia or a Manifestation of Primary Cutaneous Marginal Zone Lymphoma?

It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits. Clinical investigations excluded relevant systemic disease in all cases. Microscopically, dermal/subcutaneous deposits of amyloid were associated with sparse to moderate perivascular infiltrates of lymphocytes and monotypic plasma cells (7 with kappa and 3 with lambda light chain restriction). The plasma cells expressed CD56 in one of 9 studied cases. One case was characterized by a t(14;18)(q32;q21)/IGH-MALT1 translocation. Follow-up was available in 8 cases. All remain systemically well after a median time of 86.5 months (range: 40 to 144 mo). Local recurrence of disease was observed in 3 patients. A fourth patient presented with a cMZL without amyloid deposits 8 years after excision of the cutaneous AL amyloidoma. Although our series is small, careful categorization and follow-up of the cases, together with updated information in the literature, show clinical and biological links between AL amyloidomas of the skin/subcutis and cMZL, suggesting that at least a subset of cutaneous AL amyloidoma may represent an unusual manifestation of cMZL (cutaneous mucosa-associated lymphoid tissue lymphomas).