-
Journal of the American Veterinary... Aug 2018
Topics: Animals; Cat Diseases; Cats; Diagnosis, Differential; Escherichia coli; Escherichia coli Infections; Female; Panniculitis; Pedigree; Tail; Tinea
PubMed: 30020009
DOI: 10.2460/javma.253.3.287 -
International Journal of Rheumatic... Apr 2023"Retractile mesenteritis" was the first name given to a rare, benign, inflammatory disease that affects the adipose tissue of the intestinal mesentery and less...
"Retractile mesenteritis" was the first name given to a rare, benign, inflammatory disease that affects the adipose tissue of the intestinal mesentery and less frequently other locations. Now labeled as mesenteric panniculitis, the pathogenic mechanism remains unclear. Several stimuli could be involved, and it is sometimes associated with other conditions such as malignancy or autoimmune diseases. We present a case of mesenteric panniculitis with extensive abdominal and extra-abdominal involvement that developed a few months after SARS-COV2 infection, raising the hypothesis of this virus as a potential trigger for autoinflammatory and autoimmune diseases.
Topics: Humans; Panniculitis, Peritoneal; RNA, Viral; Diagnosis, Differential; COVID-19; SARS-CoV-2; Panniculitis
PubMed: 36565466
DOI: 10.1111/1756-185X.14544 -
Journal of Clinical Pathology Dec 2015The diagnosis of panniculitis is a relatively rare occurrence for many practising pathologists. The smaller subset of lymphocyte-predominant panniculitis is further... (Review)
Review
The diagnosis of panniculitis is a relatively rare occurrence for many practising pathologists. The smaller subset of lymphocyte-predominant panniculitis is further complicated by the diagnostic consideration of T cell lymphoma involving the subcutaneous tissue, mimicking inflammatory causes of panniculitis. Accurate classification of the panniculitis is crucial to direct clinical management as treatment options may vary from non-medical therapy to immunosuppressive agents to aggressive chemotherapy. Many diseases show significant overlap in clinical and histological features, making the process of determining a specific diagnosis very challenging. However, with an adequate biopsy including skin and deep subcutaneous tissue, a collaborative effort between clinician and pathologist can often lead to a specific diagnosis. This review provides an algorithmic approach to the diagnosis of lymphocyte-predominant panniculitis, including entities of septal-predominant pattern panniculitis (erythema nodosum, deep necrobiosis lipoidica, morphea profunda and sclerosing panniculitis) and lobular-predominant pattern panniculitis (lupus erythematous panniculitis/lupus profundus, subcutaneous panniculitis-like T cell lymphoma, cutaneous γ-δ T cell lymphoma, Borrelia infection and cold panniculitis).
Topics: Algorithms; Diagnosis, Differential; Humans; Lymphocytes; Lymphoma, T-Cell, Cutaneous; Panniculitis; Skin; Skin Neoplasms; Subcutaneous Tissue
PubMed: 26602413
DOI: 10.1136/jclinpath-2014-202849 -
BMJ Case Reports Sep 2023A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a...
A young male presented with intermittent high-grade fever, asymmetric polyarthritis and erythematous, tender nodules over left shin for 2 months duration. He had a history of alcohol dependence with multiple episodes of acute pancreatitis. With polyarthritis progressing relentlessly, unresponsive to non-steroidal anti-inflammatory drugs and steroids, a provisional diagnosis of sarcoidosis was considered. Indeed, he was treated with azathioprine and rituximab with no effect. Biopsy of the skin nodule revealed subcutaneous fat necrosis, foam cells, deposition of eosinophilic amorphous material and calcification. Synovial fluid aspiration from the arthritic knee obtained purulent but surprisingly culture-negative material, rich in triglycerides. Abdominal CT confirmed chronic pancreatitis. Final diagnosis of pancreatitis, panniculitis and polyarthritis (PPP) syndrome was made. He underwent surgical pancreatic ductal drainage leading to complete remission of symptoms. PPP syndrome triad occurs due to leakage of pancreatic enzymes into systemic circulation and subsequent fat necrosis. Surgical drainage of pancreatic duct is often curative.
Topics: Humans; Male; Pancreatitis; Acute Disease; Panniculitis; Arthritis; Subcutaneous Fat; Fat Necrosis
PubMed: 37751979
DOI: 10.1136/bcr-2023-254732 -
Archives of Pathology & Laboratory... Oct 2014Neutrophilic panniculitis encompasses an etiologically and morphologically heterogeneous group of disorders. Correct histopathologic diagnosis is important in... (Review)
Review
CONTEXT
Neutrophilic panniculitis encompasses an etiologically and morphologically heterogeneous group of disorders. Correct histopathologic diagnosis is important in identifying certain systemic diseases and guiding appropriate treatment.
OBJECTIVE
To review the clinical and histopathologic features of different types of neutrophilic panniculitis, and to provide a diagnostic algorithm for these disorders.
DATA SOURCES
A review of the literature with emphasis on the distinguishing features of different entities was performed.
CONCLUSIONS
Evaluation for neutrophilic panniculitis entails paying close attention to the pattern of inflammation, the type of fat necrosis present, any evidence of vascular damage, and other relevant histopathologic features. An algorithmic approach integrating all histopathologic, clinical, and laboratory findings is required for correct diagnosis.
Topics: Adipose Tissue; Arthritis, Rheumatoid; Decision Trees; Diagnosis, Differential; Erythema Induratum; Erythema Nodosum; Fat Necrosis; Humans; Neutrophils; Pancreatitis; Panniculitis; Subcutaneous Fat; Sweet Syndrome; Vasculitis; alpha 1-Antitrypsin Deficiency
PubMed: 25268197
DOI: 10.5858/arpa.2014-0270-CC -
Radiology May 2023
Topics: Humans; Panniculitis; Arthritis; Pancreatitis; Diagnostic Imaging
PubMed: 37129486
DOI: 10.1148/radiol.222426 -
Clinical Immunology (Orlando, Fla.) Oct 2023Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous non-Hodgkin lymphoma involving CD8 T cells, the genetic underpinnings of which remain...
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous non-Hodgkin lymphoma involving CD8 T cells, the genetic underpinnings of which remain incompletely understood. Here we report two unrelated patients with B cell Expansion with NF-κB and T cell Anergy (BENTA) disease and a novel presentation of SPTCL. Patient 1 presented early in life with recurrent infections and B cell lymphocytosis, linked to a novel gain-of-function (GOF) CARD11 mutation (p.Lys238del). He developed SPTCL-like lesions and membranoproliferative glomerulonephritis by age 2, treated successfully with cyclosporine. Patient 2 presented at 13 months with splenomegaly, lymphadenopathy, and SPTCL with evidence of hemophagocytic lymphohistiocytosis. Genetic analysis revealed two in cis germline GOF CARD11 variants (p.Glu121Asp/p.Gly126Ser). Autologous bone marrow transplant resulted in SPTCL remission despite persistent B cell lymphocytosis. These cases illuminate an unusual pathological manifestation for BENTA disease, suggesting that CARD11 GOF mutations can manifest in cutaneous CD4and CD8 T cell malignancies.
Topics: Male; Humans; Child, Preschool; CD8-Positive T-Lymphocytes; Lymphocytosis; Panniculitis; Lymphoma, T-Cell; Immunologic Deficiency Syndromes
PubMed: 37562721
DOI: 10.1016/j.clim.2023.109732 -
BMJ Case Reports Oct 2023Pancreatic panniculitis is a rare cutaneous manifestation of pancreatic disease with only scant case reports available to guide management. In this report, a woman in...
Pancreatic panniculitis is a rare cutaneous manifestation of pancreatic disease with only scant case reports available to guide management. In this report, a woman in her 60s developed a painful, erythematous and indurated, nodular rash after an episode of acute pancreatitis postendoscopic retrograde cholangiopancreatography (ERCP). While clinically and radiologically the pancreatitis improved with standard conservative management, the panniculitis remained severely debilitating. Repeat testing of serum pancreatic enzymes revealed persistent and marked elevation. Octreotide was started to inhibit pancreatic enzyme release, and the lesions improved, with resolution of the panniculitis correlating with falling serum pancreatic enzyme levels. Hence, serial pancreatic enzyme testing may have utility in monitoring and management of pancreatic panniculitis.
Topics: Female; Humans; Acute Disease; Lipase; Pancreas; Pancreatitis; Panniculitis; Aged
PubMed: 37899080
DOI: 10.1136/bcr-2022-254370 -
Clinical Journal of Gastroenterology Apr 2017Sclerosing mesenteritis includes a spectrum of inflammatory disorders involving the adipose tissue of the bowel mesentery. (Review)
Review
INTRODUCTION
Sclerosing mesenteritis includes a spectrum of inflammatory disorders involving the adipose tissue of the bowel mesentery.
AIM
To perform a systematic review of previously reported cases of sclerosing mesenteritis (SM) to determine the epidemiology, risk factors, methods of diagnosis, treatment patterns and outcomes for this disease.
METHODS
Medline, PubMed, Google Scholar and Cochrane database were searched using keywords mesenteric panniculitis, retractile mesenteritis, mesenteric lipodystrophy and sclerosing mesenteritis. Data was collated into a single excel database, transferred into SPSS (Version 21.0) and analyzed.
RESULTS
Patients diagnosed with SM were between ages of 3 and 88 with a mean age of 55 ± 19.2 years. SM was more common in Caucasians (n = 28, 60.8% of those reporting ethnicity) and men (n = 133, 69.3%) with a male to female ratio of 2.3:1. 28.6% (n = 55) of patients reported a prior abdominal surgery or abdominal trauma, 8.9% (n = 17) had a history of malignancy, and 5.7% (n = 11) of autoimmune disease. 85.4% (n = 164) underwent surgical abdominal exploration (open or laparoscopic); 41.7% (n = 80) had surgery with resection of the involved bowel and mesentery. 34.9% (n = 67) of patients received medical treatment with the majority of them receiving steroids (n = 56, 83.5%). Symptom duration of more than a month (66.7% vs 40.4%, p < 0.05), underlying autoimmune disorder (14.3% vs 4.0%, p < 0.05) or low protein (14.3% vs 4.0%, p < 0.05) at presentation were seen more frequently in those with poor treatment response whereas patients with tender abdomen (45.0% vs 19.0%, p < 0.05) or leukocytosis (20.5% vs 0.0%, p < 0.05) at presentation were likely to have good response to therapy. The most common complications included bowel obstruction/ileus/ischemia (n = 10, 23.8%) and obstructive uropathy/renal failure (n = 10, 23.8%). There were a total of 14 deaths, 12 (85.7%) of which were secondary to SM related complications.
CONCLUSION
SM is a poorly understood chronic inflammatory disease. Our study is the first systematic review of the published cases of SM. Future work is required to better understand this disease and its optimal therapy.
Topics: Humans; Lipodystrophy; Mesentery; Panniculitis, Peritoneal; Prognosis; Terminology as Topic
PubMed: 28197781
DOI: 10.1007/s12328-017-0716-5 -
International Journal of Dermatology Jul 2023VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations.
BACKGROUND
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease with frequent cutaneous manifestations.
METHODS
We conducted a retrospective study of all patients with genetically confirmed VEXAS syndrome seen at our institution. Available clinical photographs and skin biopsy slides were reviewed.
RESULTS
Cutaneous manifestations developed in 22/25 (88%) patients with VEXAS syndrome. From this group, 10/22 (45%) developed skin involvement before or at the time of other clinical features of VEXAS. Twenty distinct dermatologic presentations of VEXAS from 14 patients were reviewed, and histopathologic patterns were classified as follows: neutrophilic urticarial dermatosis (n = 5, 25%), leukocytoclastic/urticarial vasculitis (n = 4, 20%), urticarial tissue reaction (n = 4, 20%), neutrophilic dermatosis (n = 3, 15%), neutrophilic panniculitis (n = 2, 10%), and nonspecific chronic septal panniculitis (n = 2, 10%). Common systemic findings included macrocytic anemia (96%), fever (88%), thrombocytopenia (76%), weight loss (76%), ocular inflammation (64%), pulmonary infiltrates (56%), deep venous thrombosis or pulmonary embolism (52%), and inflammatory arthritis (52%).
CONCLUSIONS
Cutaneous involvement is a common feature of VEXAS syndrome, and histopathologic findings exist on a spectrum of neutrophilic inflammatory dermatoses.
Topics: Humans; Retrospective Studies; Skin; Urticaria; Dermatitis; Panniculitis; Mutation
PubMed: 36890121
DOI: 10.1111/ijd.16635