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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 -
European Journal of Radiology Oct 2023To analyze the computed tomography (CT) findings of idiopathic mesenteric panniculitis and the factors related to its characteristics and to improve the understanding of...
OBJECTIVES
To analyze the computed tomography (CT) findings of idiopathic mesenteric panniculitis and the factors related to its characteristics and to improve the understanding of the disease.
METHODS
The imaging findings of 121 patients with mesenteric panniculitis were retrospectively analyzed, along with related factors such as age, sex, and abdominal visceral fat area.
RESULTS
Among the 121 patients, two had midgut malrotation, and the lesions were located outside the mesentery on the right side of the abdominal cavity, while the lesions in the other patients were located around the mesentery on the left side of the abdominal cavity, presenting as patchy or patchy fuzzy high-density masses. Annulus fibrosus and/or fatty ring signs were also observed in some patients. Scattered soft tissue nodules were observed around the mesentery in 119 patients. Eight patients had intestinal tract traction and retraction, and one patient had secondary intestinal obstruction. Nearly half of the patients had mesenteric vascular changes, and three had mesenteric vascular thrombosis. Among the 121 patients, there were 89 males and 32 females, aged 22-83, with an average age of 52.14 ± 13.53 years. The distribution range of abdominal visceral fat area (VFA) in 121 patients was 79.85-331.65 cm.
CONCLUSION
Mesenteric panniculitis has certain characteristic imaging findings that can be accompanied by often ignored changes in the mesenteric blood vessels and intestinal tubes. Intestinal obstruction and mesenteric vascular thrombosis are rare complications, and their primary causes are often overlooked. Mesenteric panniculitis was correlated with sex, age, and VFA.
Topics: Female; Male; Humans; Adult; Middle Aged; Aged; Panniculitis, Peritoneal; Retrospective Studies; Intestinal Obstruction; Tomography, X-Ray Computed; Thrombosis
PubMed: 37666075
DOI: 10.1016/j.ejrad.2023.111071 -
Journal of Investigative Medicine High... 2024Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is... (Review)
Review
Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.
Topics: Humans; Male; Biopsy; Diagnosis, Differential; Fatal Outcome; Lymphohistiocytosis, Hemophagocytic; Lymphoma, T-Cell; Lymphoma, T-Cell, Cutaneous; Panniculitis; Skin; Skin Neoplasms; Young Adult
PubMed: 38742532
DOI: 10.1177/23247096241253337 -
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 -
Terapevticheskii Arkhiv Jun 2020To study clinical and laboratory features of panniculitis (Pn) in modern rheumatology.
AIM
To study clinical and laboratory features of panniculitis (Pn) in modern rheumatology.
MATERIALS AND METHODS
The study included 690 patients with Pn (615 women and 75 men, average age 39.410.26) with the prevailing referral diagnosis of Erythema nodosum (59.2%), Pn (27.5%), Rheumatic disease Rd (9%), other diseases (4.4%),who had been on outpatient and/or inpatient treatment for 10 years. All patients were examined according to our diagnostic algorithm: general clinical, immunological and histological examinations, computed tomography of the chest organs, tuberculin tests.
RESULTS
Pn with Rd was diagnosed in 140 patients (118 women and 22 men, average age 40.2114.87), average disease duration 31.06 [0.1; 541] months. In most cases (49%) patients had idiopathic lobular Pn which belongs to the group of systematic lesions of connective tissue (M35.6), as well as systemic lupus erythematosus and Behcet disease (13% each), rheumatoid arthritis (8%), dermatomyositis (6.4%), etc. Matching of the referral and final diagnoses was 35% in case of Rd. Among the examined patients prevailed those with a moderate (51.07%) degree of activity of the underlying disease. Within the study group Pn was represented by all forms, but mainly by nodular form (64.02%). Mesenteric form was characteristic only for idiopathic lobular Pn. The main features of Pn associated with Rd were identified. In modern clinical practice the type of Pn and the activity of the underlying disease determine the approaches to treatment.
Topics: Child; Child, Preschool; Erythema Nodosum; Female; Humans; Lupus Erythematosus, Systemic; Male; Panniculitis; Rheumatic Diseases; Rheumatology
PubMed: 32598773
DOI: No ID Found -
Cureus Jan 2022Coronavirus disease 2019 (COVID-19) typically involves the respiratory system, but gastrointestinal involvement is common. Further, patients with severe COVID-19 are at...
Coronavirus disease 2019 (COVID-19) typically involves the respiratory system, but gastrointestinal involvement is common. Further, patients with severe COVID-19 are at high risk to develop gastrointestinal complications, including bowel ischemia, ileus, and deranged liver enzymes. We present the case of a 44-year-old woman with mild COVID-19 pneumonia who was in home isolation. Ten days after the isolation, the patient presented to the emergency department complaining of generalized abdominal pain that was sharp in nature and associated with nausea and recurrent episodes of vomiting. The patient did not complain of any respiratory symptoms. Physical examination showed diffuse tenderness with no clinical signs to suggest generalized peritonitis. The laboratory parameters showed normal hematological, renal, and hepatic profiles. No elevation in the inflammatory markers was observed. The amylase level was within the normal range. Abdominal computed tomography scan demonstrated the presence of misty mesentery with increased density of the mesentery with fat stranding encasing the mesenteric vessels along with mesenteric adenopathy. Such radiological features suggested the diagnosis of mesenteric panniculitis. Subsequently, intravenous corticosteroid therapy was initiated and the patient exhibited significant improvement after 24 hours. The patient was discharged after nine days of hospitalization. She was followed up after one month and she had no complaints. Mesenteric panniculitis is a rare idiopathic inflammatory condition involving the mesenteric adipose tissue. The case shed a light on the possible association of COVID-19 with mesenteric panniculitis. The clinical manifestations of mesenteric panniculitis are non-specific and imaging studies are essential to suggest the diagnosis.
PubMed: 35186573
DOI: 10.7759/cureus.21314 -
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 -
Clinical and Experimental Dermatology Mar 2020
Review
Topics: Diagnosis, Differential; Female; Humans; Immunosuppressive Agents; Microscopic Polyangiitis; Middle Aged; Panniculitis; Renal Insufficiency; Skin
PubMed: 30932212
DOI: 10.1111/ced.13912