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Acta Bio-medica : Atenei Parmensis Dec 2019The mesenteric panniculitis is a rare form of inflammation that mainly involves the mesenteric adipose tissue. The etiology remains unknown and the disease has been... (Review)
Review
The mesenteric panniculitis is a rare form of inflammation that mainly involves the mesenteric adipose tissue. The etiology remains unknown and the disease has been associated with various conditions such as cancer, abdominal trauma, previous surgery, autoimmune diseases and obesity. Mesenteric panniculitis can be divided into two main groups: the mesenteric panniculitis with only the inflammation and degeneration of the mesenteric fat, and the retractile panniculitis, mainly fibrotic, with retraction of the surrounding structures. From a radiological point of view, there are two main signs: the fat ring sign, which is the presence of normal fat around vessels and lymph nodes, and the pseudocapsula around the lesion. In this paper, we present the imaging and clinical features of mesenteric panniculits with particular reference to the differential diagnosis and the possible etiological associations. (www.actabiomedica.it).
Topics: Humans; Magnetic Resonance Imaging; Panniculitis, Peritoneal; Tomography, X-Ray Computed
PubMed: 31910164
DOI: 10.23750/abm.v90i4.7696 -
Journal of the American Academy of... Oct 2020Antineoplastic agents that use the immune system have revolutionized cancer treatment. Specifically, implementation of immune checkpoint inhibitors, monoclonal... (Review)
Review
Antineoplastic agents that use the immune system have revolutionized cancer treatment. Specifically, implementation of immune checkpoint inhibitors, monoclonal antibodies that block cytotoxic T-lymphocyte-associated antigen-4, programmed cell death protein 1, or programmed cell death ligand 1 show improved and sustained responses in patients with cancer. However, these agents are associated with a plethora of adverse events, many manifesting in the skin. As the clinical application of cancer immunotherapies expands, understanding the clinical and histopathologic features of associated cutaneous toxicities becomes increasingly important to dermatologists, oncologists, and pathologists to ensure timely diagnosis and appropriate care. This review discusses cutaneous reactions to immune checkpoint inhibitors, focusing on histopathologic features.
Topics: Acantholysis; Alopecia; Drug Eruptions; Humans; Immune Checkpoint Inhibitors; Keratinocytes; Lichenoid Eruptions; Nevus, Pigmented; Panniculitis; Pemphigoid, Bullous; Pruritus; Psoriasis; Stevens-Johnson Syndrome; Vitiligo
PubMed: 32360716
DOI: 10.1016/j.jaad.2020.04.105 -
Gastroenterology & Hepatology Apr 2023Mesenteric panniculitis (MP) is a benign condition characterized by chronic inflammation and fibrosis of adipose tissue mainly of the small bowel mesentery. MP is...
Mesenteric panniculitis (MP) is a benign condition characterized by chronic inflammation and fibrosis of adipose tissue mainly of the small bowel mesentery. MP is commonly detected incidentally on cross-sectional imaging of the abdomen and can be asymptomatic in up to nearly half of patients. The most frequent clinical symptom reported is abdominal pain, followed by bloating/distention, diarrhea, constipation, vomiting, anorexia, weight loss, fever, malaise, and nausea. On computed tomography, MP is seen as a mass-like area of increased fat attenuation within the small bowel mesentery, usually located in the left upper quadrant of the abdomen. This mass-like area envelops mesenteric vessels and displaces adjacent bowel segments. Lymph nodes are frequently seen within the area of mesenteric abnormality. One of the most common differential diagnoses of MP is lymphoma, and positron emission tomography/computed tomography may be performed if there is suspicion of a concurrent underlying malignancy. Because of the benign nature of MP, treatment decisions should be guided by severity of symptoms and presence of complications. First-line medical treatment is prednisone and tamoxifen. Surgery is reserved for cases of recurrent bowel obstruction. This article provides a review of MP, including its epidemiology, pathophysiology, clinical presentation, imaging findings, and treatment.
PubMed: 37705847
DOI: No ID Found -
The Journal of Clinical Investigation Apr 2020BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine... (Clinical Trial)
Clinical Trial
BACKGROUNDUndifferentiated systemic autoinflammatory diseases (USAIDs) present diagnostic and therapeutic challenges. Chronic interferon (IFN) signaling and cytokine dysregulation may identify diseases with available targeted treatments.METHODSSixty-six consecutively referred USAID patients underwent underwent screening for the presence of an interferon signature using a standardized type-I IFN-response-gene score (IRG-S), cytokine profiling, and genetic evaluation by next-generation sequencing.RESULTSThirty-six USAID patients (55%) had elevated IRG-S. Neutrophilic panniculitis (40% vs. 0%), basal ganglia calcifications (46% vs. 0%), interstitial lung disease (47% vs. 5%), and myositis (60% vs. 10%) were more prevalent in patients with elevated IRG-S. Moderate IRG-S elevation and highly elevated serum IL-18 distinguished 8 patients with pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS). Among patients with panniculitis and progressive cytopenias, 2 patients were compound heterozygous for potentially novel LRBA mutations, 4 patients harbored potentially novel splice variants in IKBKG (which encodes NF-κB essential modulator [NEMO]), and 6 patients had de novo frameshift mutations in SAMD9L. Of additional 12 patients with elevated IRG-S and CANDLE-, SAVI- or Aicardi-Goutières syndrome-like (AGS-like) phenotypes, 5 patients carried mutations in either SAMHD1, TREX1, PSMB8, or PSMG2. Two patients had anti-MDA5 autoantibody-positive juvenile dermatomyositis, and 7 could not be classified. Patients with LRBA, IKBKG, and SAMD9L mutations showed a pattern of IRG elevation that suggests prominent NF-κB activation different from the canonical interferonopathies CANDLE, SAVI, and AGS.CONCLUSIONSIn patients with elevated IRG-S, we identified characteristic clinical features and 3 additional autoinflammatory diseases: IL-18-mediated PAP and recurrent MAS (IL-18PAP-MAS), NEMO deleted exon 5-autoinflammatory syndrome (NEMO-NDAS), and SAMD9L-associated autoinflammatory disease (SAMD9L-SAAD). The IRG-S expands the diagnostic armamentarium in evaluating USAIDs and points to different pathways regulating IRG expression.TRIAL REGISTRATIONClinicalTrials.gov NCT02974595.FUNDINGThe Intramural Research Program of the NIH, NIAID, NIAMS, and the Clinical Center.
Topics: Autoimmune Diseases; Female; Humans; Interferon Type I; Interleukin-18; Macrophage Activation Syndrome; Male; Mutation; Panniculitis; Pulmonary Alveolar Proteinosis
PubMed: 31874111
DOI: 10.1172/JCI129301 -
JAMA Dermatology Nov 2020This case series characterizes the initial presentation and treatment of lupus erythematosus panniculitis/profundus and its association with systemic lupus erythematosus...
This case series characterizes the initial presentation and treatment of lupus erythematosus panniculitis/profundus and its association with systemic lupus erythematosus and discoid lupus erythematosus.
Topics: Adolescent; Adult; Aged; Antirheumatic Agents; Child; Child, Preschool; Comorbidity; Delayed Diagnosis; Female; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Male; Middle Aged; Panniculitis, Lupus Erythematosus; Retrospective Studies; Young Adult
PubMed: 32876656
DOI: 10.1001/jamadermatol.2020.2797 -
Journal of the American Academy of... Oct 2022Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports... (Review)
Review
BACKGROUND
Panniculitis represents a rare and potentially lethal manifestation of alpha-1 antitrypsin deficiency (AATD). Evidence regarding management is limited to case reports and small case series. We sought to clarify typical features and investigation of AATD-associated panniculitis and assess the evidence regarding therapeutic options.
SEARCH METHODOLOGY
Articles and abstracts published between 1970 and 2020 were identified by searches of MEDLINE, PubMed, and secondary searches of references from relevant articles using the search terms "panniculitis," "alpha-1," "antitrypsin," "deficiency," and "Weber-Christian."
FINDINGS
We identified 117 cases of AATD-associated panniculitis. In 1 series, AATD was present in 15% of all cases of biopsy-proven panniculitis. Failure to achieve clinical response was seen in all instances of systemic steroid use. Dapsone, although effective and accessible, is frequently associated with failure to achieve remission. In these instances, intravenous AAT augmentation therapy generally resulted in response.
CONCLUSIONS
AATD may be more prevalent among patients presenting with panniculitis than previously thought. Patients presenting with panniculitis and systemic illness show high mortality risk. Although most cases are associated with the severe ZZ-genotype, moderate genotypes may also predispose to panniculitis. Dapsone remains the most cost-effective therapeutic option, whereas intravenous AAT augmentation remains the most efficacious. Finally, glucocorticoids appear ineffective in this setting.
Topics: Dapsone; Glucocorticoids; Humans; Panniculitis; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 33516773
DOI: 10.1016/j.jaad.2021.01.074 -
Dermatopathology (Basel, Switzerland) Aug 2021Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many... (Review)
Review
Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many aetiologies. As in adults, the panniculitis can be the primary process in a systemic disorder or a secondary process that results from infection, trauma or exposure to medication. Some types of panniculitis are seen more commonly or exclusively in children, and several new entities have been described in recent years. Most types of panniculitis have the same clinical presentation (regardless of the aetiology), with tender, erythematous subcutaneous nodules. Although the patient's age and the lesion site provide information, a histopathological assessment is sometimes required for a definitive diagnosis and classification of the disorder. In children, most panniculitides are lobular. At present, autoimmune inflammatory diseases and primary immunodeficiencies have been better characterised; panniculitis can be the presenting symptom in some of these settings. Unexplained panniculitis in a young child should prompt a detailed screen for monogenic immune disorders because the latter usually manifest themselves early in life. Here, we review forms of panniculitis that occur primarily in children, with a focus on newly described entities.
PubMed: 34449587
DOI: 10.3390/dermatopathology8030037 -
Clinics in Dermatology 2020Neutrophilic drug reactions are unique eruptions that can affect hospitalized patients and share a common pathophysiology with neutrophils as the key mediators of... (Review)
Review
Neutrophilic drug reactions are unique eruptions that can affect hospitalized patients and share a common pathophysiology with neutrophils as the key mediators of inflammation. They range in clinical presentation from papules and plaques to bullae and erosions to pustules. Although there is some overlap in presentation, each has distinguishing features that aid the clinician in differentiation from one another and from other drug hypersensitivity reactions. Much of the data on these reactions are from case reports and series or retrospective review studies. There are limited prospective observational studies dedicated to these adverse drug reactions. We review the more common and life-threatening neutrophilic drug reactions, their proposed mechanism of action, and their management.
Topics: Drug Eruptions; Drug Hypersensitivity; Drug-Related Side Effects and Adverse Reactions; Exanthema; Female; Halogens; Hidradenitis; Humans; Immunoglobulin A; Male; Neutrophils; Panniculitis; Pharmaceutical Preparations; Pyoderma Gangrenosum; Skin Diseases, Vesiculobullous; Sweet Syndrome
PubMed: 33341199
DOI: 10.1016/j.clindermatol.2020.06.012 -
La Revue de Medecine Interne Nov 2023
Topics: Humans; Skin Diseases; Panniculitis; Skin
PubMed: 37414675
DOI: 10.1016/j.revmed.2023.06.006