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American Journal of Clinical Dermatology May 2021Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact... (Review)
Review
Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact cause of erythema nodosum is unknown, although it appears to be a hypersensitivity response to a variety of antigenic stimuli. Although the etiology is mostly idiopathic, ruling out an underlying disease is imperative before diagnosing primary erythema nodosum. Erythema nodosum can be the first sign of a systemic disease that is triggered by a large group of processes, such as infections, inflammatory diseases, neoplasia, and/or drugs. The most common identifiable causes are streptococcal infections, primary tuberculosis, sarcoidosis, Behçet disease, inflammatory bowel disease, drugs, and pregnancy. We propose a diagnostic algorithm to optimize the initial work-up, hence initiating prompt and accurate management of the underlying disease. The algorithm includes an initial assessment of core symptoms, diagnostic work-up, differential diagnosis, and recommended therapies. Several treatment options for the erythema nodosum lesions have been previously reported; nevertheless, these options treat the symptoms, but not the triggering cause. Making an accurate diagnosis will allow the physician to treat the underlying cause and determine an optimal therapeutic strategy.
Topics: Anti-Inflammatory Agents; Diagnosis, Differential; Drug Therapy, Combination; Erythema Nodosum; Humans; Incidence; Skin; Treatment Outcome
PubMed: 33683567
DOI: 10.1007/s40257-021-00592-w -
Anais Brasileiros de Dermatologia 2023Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple... (Review)
Review
Cutaneous lupus erythematosus is an autoimmune disease of varied clinical expression, which may present as an exclusively cutaneous disease or be one of the multiple manifestations of systemic lupus erythematosus. Its classification includes acute, subacute, intermittent, chronic and bullous subtypes, which are usually identified based on clinical features and histopathological and laboratory findings. Other non-specific cutaneous manifestations may be associated with systemic lupus erythematosus and are usually related to disease activity. Environmental, genetic and immunological factors play a role in the pathogenesis of skin lesions in lupus erythematosus. Recently, considerable progress has been made in elucidating the mechanisms involved in their development, which allows for foreseeing future targets for more effective treatments. This review proposes to discuss the main etiopathogenic, clinical, diagnostic and therapeutic aspects of cutaneous lupus erythematosus, aiming to update internists and specialists from different areas.
Topics: Humans; Lupus Erythematosus, Discoid; Lupus Erythematosus, Cutaneous; Lupus Erythematosus, Systemic; Autoimmune Diseases; Skin
PubMed: 36868923
DOI: 10.1016/j.abd.2022.09.005 -
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 -
Animals : An Open Access Journal From... Oct 2022Acute phase response is a nonspecific reaction to disturbances in homeostasis during which the production of some Acute Phase Proteins (APPs) is stimulated; they are... (Review)
Review
Acute phase response is a nonspecific reaction to disturbances in homeostasis during which the production of some Acute Phase Proteins (APPs) is stimulated; they are sensitive but nonspecific markers of systemic inflammatory processes. The major positive APP in dogs is the C-reactive protein (CRP). The dynamic of its concentration changes fast, rising and decreasing rapidly with the onset and removal of the inflammatory stimulus. It increases within the first 4-24 h after the stimulus and reaches up to a 50-100-fold increase of the baseline level. It has been documented that this APP's concentration is elevated during several diseases, such as pyometra, panniculitis, acute pancreatitis, polyarthritis, sepsis, immune-mediated hemolytic anemia, and neoplasia in dogs. In clinical practice, canine CRP is mostly measured to detect and monitor systemic inflammatory activity and the efficacy of treatments, because it is a more sensitive marker than shifts in leukocyte counts. Blood serum CRP concentration is becoming a part of routine biochemistry panels in many countries. In this article, changes in CRP concentration and its clinical application in healthy and diseased dogs are discussed.
PubMed: 36290272
DOI: 10.3390/ani12202888 -
Clinics in Colon and Rectal Surgery Jul 2022Mesenteric panniculitis (MP) is the preferred nomenclature for a continuum of inflammatory diseases of the mesentery. The diagnosis of MP is often based on the... (Review)
Review
Mesenteric panniculitis (MP) is the preferred nomenclature for a continuum of inflammatory diseases of the mesentery. The diagnosis of MP is often based on the appearance of a mass-like structure at the root of the mesentery. Characteristic histology includes focal fat necrosis, chronic inflammation, and sometimes mesenteric fibrosis. At present, robust literature related to diagnosis and management of MP are limited. MP is postulated to be an immune-mediated chronic inflammatory and/or a paraneoplastic disease. A personal or family history of other autoimmune diseases is commonly apparent. Several inciting events have been identified that possibly act as triggers in the development of the disease. Trauma, abdominal surgery, infection, and various cancers have been associated with mesenteric panniculitis. There are several diagnostic and histologic criteria that aid in making the diagnosis of MP. The differential diagnosis for a mesenteric mass includes neoplastic disease, and a biopsy may be indicated to rule out other conditions. While cases of MP with a short duration of symptoms, or spontaneously regression may occur, some patients experience prolonged periods of pain, fever, and alterations in bowel habit, causing significant morbidity. A variety of medical therapies have been suggested for MP. Only two, thalidomide and low-dose naltrexone, have been prospectively evaluated. For patients with chronic MP, good responses to prolonged corticosteroid treatment have been reported. Novel therapies include thalidomide and low-dose naltrexone. Hormonal and immunomodulatory therapies are also used based on small case series, but these treatments may have significant side effects. Surgical intervention is not curative and is avoided except for relief of focal bowel obstruction secondary to fibrotic forms of the disease.
PubMed: 35966977
DOI: 10.1055/s-0042-1743588 -
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