-
The American Journal of Surgical... Dec 2023Distinguishing lupus erythematosus panniculitis (LEP) from subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a diagnostic challenge with important clinical...
Distinguishing lupus erythematosus panniculitis (LEP) from subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a diagnostic challenge with important clinical implications. Immunohistochemical expression of interferon regulatory factor 8 (IRF8) has been shown to highlight cells with plasmacytoid dendritic cell differentiation. Considering that the presence of plasmacytoid dendritic cells highlighted by CD123 immunolabeling is a well-described feature that supports LEP over SPTCL, we hypothesized that IRF8 immunohistochemistry can be used as a diagnostic test to improve accuracy in differentiating LEP from SPTCL. In this study, we assessed the expression of IRF8, CD123, and CD20 in 35 cutaneous biopsies from 31 distinct patients, which included 22 cases of LEP and 13 cases of SPTCL. We found that clusters of IRF8-positive cells within the dermis, and away from subcutaneous fat, could discriminate LEP from SPTCL ( P =0.005). Similarly, CD123-positive clusters in any location were observed in LEP but absent in all cases of SPTCL. In addition, we found that dermal CD20-predominant lymphoid aggregates could help discriminate LEP from SPTCL ( P =0.022). As individual assays, IRF8, CD123, and CD20 were highly specific (100%, 100%, and 92%, respectively) though poorly sensitive (45%, 29%, and 50%, respectively). However, a panel combining IRF8, CD123, and CD20, with at least 1 positive marker was more accurate than any individual marker by receiver operating characteristic curve analysis. Our study provides a rationale for potentially including IRF8 as part of an immunohistochemical panel composed of other currently available markers used to differentiate LEP from SPTCL.
Topics: Humans; Panniculitis, Lupus Erythematosus; Interleukin-3 Receptor alpha Subunit; Panniculitis; Interferon Regulatory Factors
PubMed: 37767989
DOI: 10.1097/PAS.0000000000002133 -
Sichuan Da Xue Xue Bao. Yi Xue Ban =... Dec 2019To explore the similarities and differences in clinical pathological features and gene rearrangement of lupus erythematosus profundus(LEP) and subcutaneous... (Comparative Study)
Comparative Study
OBJECTIVE
To explore the similarities and differences in clinical pathological features and gene rearrangement of lupus erythematosus profundus(LEP) and subcutaneous panniculitis-like T-cell lymphoma(SPTL).
METHODS
We compared the clinical presentations, histopathology, immunophenotypical features and T-cell receptor (TCR) gene rearrangement findings of 9 cases of LEP and SPTL.
RESULTS
For clinical features, most patients of LEP occurred on head and face without systemic symptoms. LEP patients responded well to hydroxycholorquine treatment with good prognosis. Most patients of SPTL tended to lower extremities involvement and accompanied with systemic symptoms, the patients with disseminated lesions or hemophagocytic syndrome(HPS) showed poorer prognosis. For histopathology, LEP patients showed dense inflammatory infiltrate in the dermis consisting predominantly of lymphocytes with less numbers of plasma cells. However, the dermis was spared in SPTL, and rimming of adipocytes and erythrophagocytosis was observed in SPTL. Lymphocytes of LEP expressing CD4/CD8, as well as clusters of CD20. CD138-positive cells and scatter of CD123-positive cells were also observed in LEP. Tumor cells of SPTL were CD4/CD8, βF1, CD138 and CD123. The expression of TIA-1 or GrB was more favor in SPTL. Monoclonal T-cell receptor-γ gene rearrangement was found in 89% of SPTL patients while negative for LEP.
CONCLUSION
Base on different clinical and pathological features, it is easy to distinguish LEP from SPTL. However, a minority of lesions in LEP localize at subcutaneous tissue, which may turn to immunophenotypical and TCR gene rearrangement test for diagnosis.
Topics: Humans; Lymphoma, T-Cell; Panniculitis; Panniculitis, Lupus Erythematosus; Subcutaneous Tissue
PubMed: 31880127
DOI: No ID Found -
The Journal of Dermatology Jul 2024Erythema nodosum (EN) may be idiopathic or secondary, and usually resolves naturally within 1-2 months. In atypical EN cases, the rash extends beyond the lower limbs...
Erythema nodosum (EN) may be idiopathic or secondary, and usually resolves naturally within 1-2 months. In atypical EN cases, the rash extends beyond the lower limbs to the upper limbs and trunk, and histopathological findings may be accompanied by vasculitis in addition to septal panniculitis. Few studies have examined the differences in the clinical characteristics of patients with EN based on rash distribution. We retrospectively examined whether there was a correlation with clinical information, such as the presence or absence of underlying diseases, by classifying the patients into two groups: the lower limbs group (the EN rash was confined to the lower limbs) and the beyond lower limbs group (the EN rash appeared beyond the lower limbs). Among the 86 adult patients diagnosed with EN at the Dermatology Department of Fujita Medical University between 2015 and 2020, there were 65 cases of the lower limbs group and 21 cases of the beyond lower limbs group. The frequency of underlying diseases was significantly higher in the beyond lower limbs group (76.2%, 16 cases) than in the lower limbs group (40.0%, 26 cases; P < 0.005). Vasculitis was more notable in the beyond lower limbs group (P < 0.05). Significantly higher vasculitis was noted in the EN group with underlying diseases (30.2%, 13 cases) than in the idiopathic EN group without underlying diseases (11.6%, 5 cases; P < 0.05). Neutrophil extracellular traps were positive in approximately 40% of cases in both groups. In the beyond lower limbs group, the possibility of severe cases with underlying diseases, vasculitis, and inflammation must be considered for effective treatment.
Topics: Humans; Erythema Nodosum; Retrospective Studies; Male; Female; Middle Aged; Adult; Aged; Skin; Lower Extremity; Vasculitis; Young Adult; Panniculitis; Aged, 80 and over; Adolescent
PubMed: 38507514
DOI: 10.1111/1346-8138.17195 -
Dermatology Online Journal Mar 2018Subcutaneous fat necrosis of the newborn is an uncommon, transient, and self-healing panniculitis, mostly affecting term newborns with perinatal complications. The...
Subcutaneous fat necrosis of the newborn is an uncommon, transient, and self-healing panniculitis, mostly affecting term newborns with perinatal complications. The authors present a case of a female full-term neonate, born from an uncomplicated pregnancy, admitted into the neonatology unit 5 hours after delivery because of refractory multifocal seizures in the context of hypoxic-ischemic encephalopathy. Nine days after birth, indurated and erythematous nodules and plaques were noted on the left arm and back. Skin biopsy was compatible with subcutaneous fat necrosis of the newborn. Laboratory evaluation including serum calcium showed normal values. No treatment was initiated. This entity generally follows an uncomplicated course. However, there are important complications for which the patient must be regularly monitored, including thrombocytopenia, hypoglycemia, hypertriglyceridemia, and most importantly, hypercalcemia. Patients should have serial serum calcium determinations for up to 6 months after the appearance of the skin lesions. The early diagnosis and prompt treatment of hypercalcemia are essential to prevent severe complications.
Topics: Biopsy; Fat Necrosis; Female; Humans; Hypoxia; Infant, Newborn; Panniculitis; Skin; Subcutaneous Fat
PubMed: 29634887
DOI: No ID Found -
Vnitrni Lekarstvi 2018Weber-Christian disease is a rare disease from the group of chronic fibrosing conditions characterized by inflamma-tion of the adipose tissue - panniculitis and...
Weber-Christian disease is a rare disease from the group of chronic fibrosing conditions characterized by inflamma-tion of the adipose tissue - panniculitis and fibrosing with frequent systemic manifestations. Etiopathogenesis of the disease is not fully known, participation of autoimmune mechanisms is anticipated. Here, we report a case of a patient with this rare disease, diagnosed after a long and demanding diagnostic process, including repeated lapa-rotomies. However, after immunosuppressive therapy, clinical and laboratory symptomatology improved rapidly as well as the patients quality of life. Key words: panniculitis - sclerosing mesenteritis - Weber-Christian disease.
Topics: Humans; Panniculitis, Nodular Nonsuppurative
PubMed: 30590944
DOI: No ID Found -
Nederlands Tijdschrift Voor Geneeskunde Apr 2021Diseases of the pancreas may present with extrapancreatic symptoms, such as (poly)arthritis or necrosis of subcutaneous fat. A combination of pancreatitis, panniculitis...
BACKGROUND
Diseases of the pancreas may present with extrapancreatic symptoms, such as (poly)arthritis or necrosis of subcutaneous fat. A combination of pancreatitis, panniculitis and (poly)arthritis is referred to as the PPP syndrome, which is associated with acute and chronic pancreatitis, as well as pancreatic malignancies.
CASE DESCRIPTION
This article describes a patient which was admitted to our hospital with severe polyarthritis and panniculitis. A meticulous work-up revealed an underlying focal alcoholic pancreatitis. The clinical course in our patient illustrates the severity of the PPP syndrome and emphasizes the need of a multidisciplinary approach.
CONCLUSION
Panniculitis and/or (poly)arthritis may be the first symptom of underlying pancreatic disease. Timely recognition and diagnosis is imperative for successful treatment and outcome. The multi-organ involvement in the PPP syndrome requires close collaboration across different medical departments.
Topics: Adult; Arthritis; Humans; Male; Necrosis; Pancreas; Pancreatic Neoplasms; Pancreatitis; Panniculitis; Syndrome
PubMed: 33914425
DOI: No ID Found -
Sclerosing mesenteritis and mesenteric panniculitis - clinical experience and radiological features.BMC Gastroenterology Jun 2017Sclerosing mesenteritis (SM) is sometimes used as an umbrella-term for idiopathic inflammatory conditions in the mesentery. Mesenteric panniculitis (MP) is a...
BACKGROUND
Sclerosing mesenteritis (SM) is sometimes used as an umbrella-term for idiopathic inflammatory conditions in the mesentery. Mesenteric panniculitis (MP) is a radiological finding and its relation to clinical SM is not fully understood. The aims of this study were to determine whether any correlation could be found between the radiological findings and the clinical disease course.
METHODS
Patients observed due to idiopathic inflammation of the mesentery were identified. If SM could be verified histologically or MP radiologically, the patients were included in this descriptive retro perspective study.
RESULTS
Typical radiological changes were observed in 27 patients. A majority (23/27) of these patients had mild to moderate symptoms. This group with typical radiology was labelled MP. Four patients were included due to histologically verified disease but had uncharacteristic radiology involving multiple compartments of the abdomen. All four had marked systemic inflammation, fever and fluctuating radiologic findings. Three had severe disease with multiple hospitalisations and complications but responded promptly to corticosteroids. This group was denoted SM.
CONCLUSIONS
We have identified two subgroups of patients; firstly, MP with stable and characteristic radiologic changes and secondly SM with atypical radiology and a more aggressive clinical course. We propose that the term SM should be reserved for this latter condition.
Topics: Adult; Aged; Diagnosis, Differential; Disease Progression; Female; Humans; Male; Middle Aged; Panniculitis; Panniculitis, Peritoneal; Prospective Studies; Radiography; Registries; Retrospective Studies; Sweden; Terminology as Topic
PubMed: 28610559
DOI: 10.1186/s12876-017-0632-7 -
Annales de Dermatologie Et de... Dec 2020BRAF inhibitors±MEK inhibitors can cause panniculitis. Since the initial case described in 2012 by Zimmer et al., some sixty further cases have been reported. Based on... (Review)
Review
INTRODUCTION
BRAF inhibitors±MEK inhibitors can cause panniculitis. Since the initial case described in 2012 by Zimmer et al., some sixty further cases have been reported. Based on a clinical study and a recent and complete review of the literature, we set out in detail the characteristics of panniculitis occurring during BRAF and MEK inhibition therapy as well as the treatment thereof.
PATIENTS AND METHODS
A 25-year-old-patient followed for multi-metastatic melanoma and taking dabrafenib and trametinib consulted for the appearance, twenty-two days after the start of targeted therapy (TT), of panniculitis of the legs and forearms possibly induced by the TT after other causes had been ruled out. The TT had been continued following dose reduction and corticoid therapy for ten days, and complete resolution occurred after fifteen days.
RESULTS
Fifty-three cases of panniculitis during BRAF±MEK inhibition therapy were analysed. The condition occurred mainly with BRAF inhibitors alone (especially vemurafenib), but it was also described with three combinations of BRAF and MEK inhibitors, regardless of age (median: 45 years), with a M/F ratio of 0.51, and in 50 % of cases, it occurred within the first month (time to onset: between 1 and 480 days). Non-specific biopsy is useful to rule out differential diagnoses. Symptomatic anti-inflammatory treatment, whether systemic or topical, may be given. In the absence of signs of severity, the TT may be continued.
CONCLUSION
When panniculitis occurs during BRAF±MEK inhibitor therapy, the causal role of the TT must be considered after full etiological investigation. It is essential to determine whether a causal relationship exists in order to avoid unwarranted cessation of treatment.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Humans; Infant, Newborn; Melanoma; Mitogen-Activated Protein Kinase Kinases; Oximes; Panniculitis; Protein Kinase Inhibitors; Proto-Oncogene Proteins B-raf; Skin Neoplasms
PubMed: 32948319
DOI: 10.1016/j.annder.2020.07.013 -
Mayo Clinic Proceedings May 2024Sclerosing mesenteritis (SM), an idiopathic nonneoplastic condition affecting 0.18% to 3.14% of the population, is characterized by chronic fat necrosis, inflammation,... (Review)
Review
Sclerosing mesenteritis (SM), an idiopathic nonneoplastic condition affecting 0.18% to 3.14% of the population, is characterized by chronic fat necrosis, inflammation, and fibrosis most commonly of the mesentery of the small intestine. Sclerosing mesenteritis typically presents in the fifth or sixth decade of life, where patients with a history of abdominal surgery and/or autoimmune disease may be at higher risk. While many patients are asymptomatic, clinical features and complications are related to the mass effect resulting from the inflammation and fibrosis involved in the pathogenesis of SM. When present, common signs, symptoms, and complications include abdominal pain, weight loss, diarrhea, palpable abdominal mass on examination, bowel obstruction, chylous ascites, and mesenteric vessel thrombosis. Although SM was historically diagnosed predominantly by biopsy, current practice has shifted away from this to computed tomography imaging of the abdomen, given the invasive nature of biopsy. However, certain conditions, including mesenteric neoplasia (lymphoma, metastatic carcinoid tumor, desmoid tumor, mesenteric carcinomatosis), can mimic SM on imaging, and if clinical suspicion is equivocal, a biopsy may be warranted for definitive diagnosis. Asymptomatic patients do not require treatment. For patients with pronounced symptoms or complicated SM, the combination of tamoxifen 10 mg twice daily and prednisone 40 mg daily is the first-line pharmacotherapy; no randomized controlled trial of this regimen has been performed. Rarely, surgery may be necessary in cases of persistent bowel obstruction refractory to medical management. Sclerosing mesenteritis has an overall benign course in most cases, but disease progression and fatal outcomes have been reported.
Topics: Humans; Panniculitis, Peritoneal; Diagnosis, Differential
PubMed: 38702129
DOI: 10.1016/j.mayocp.2024.01.019 -
Pediatric Rheumatology Online Journal Sep 2023Panniculitis, a type of inflammation of subcutaneous fat, is a relatively uncommon condition that usually presents as inflammatory nodules or plaques, with various...
BACKGROUND
Panniculitis, a type of inflammation of subcutaneous fat, is a relatively uncommon condition that usually presents as inflammatory nodules or plaques, with various proposed etiologic factors. The association between panniculitis and enthesitis-related arthritis has not been described previously.
CASE PRESENTATION
Herein, we describe a case of a 11-year-old girl who presented with recurrent fever and painful subcutaneous nodules on her extremities and buttocks. Histological examination of the skin biopsy specimen revealed lobular panniculitis. Despite the use of prednisone and mycophenolate mofetil for several months, the patient experienced a relapse of skin lesions and additional symptoms of peripheral joint swelling and inflammatory lumbar pain. She was diagnosed with enthesitis-related arthritis after confirmation by imaging. The panniculitis demonstrated a sustained response when a tumor necrosis factor alpha inhibitor was used for enthesitis-related arthritis. At 2-year follow-up, her skin lesions and arthritis remained stable.
CONCLUSIONS
Although rare, panniculitis can be considered an unusual extra-articular manifestation of enthesitis-related arthritis based on clinical and pathological insights.
Topics: Female; Humans; Child; Arthritis, Juvenile; Panniculitis; Inflammation; Mycophenolic Acid; Pain
PubMed: 37697374
DOI: 10.1186/s12969-023-00888-7