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Clinical Rheumatology Apr 2021A 42-year-old Caucasian female presented with lower limb panniculitis and bilateral ankle arthritis in the absence of abdominal or other localizing symptoms. Abdominal... (Review)
Review
A 42-year-old Caucasian female presented with lower limb panniculitis and bilateral ankle arthritis in the absence of abdominal or other localizing symptoms. Abdominal imaging revealed subacute pancreatitis with pseudocyst formation. The clinical manifestations were compatible with pancreatitis, panniculitis, and polyarthritis syndrome (PPP syndrome), a very rare complication of pancreatic disease. The patient improved with conservative treatment for the pancreatic disease and systemic corticosteroids for the cutaneous and articular manifestations. We identified 59 patients with the PPP syndrome from the literature, the majority of patients being male (74.6%) with a median age of 49 (IQR 41-63.5) years. Acute pancreatitis is the most frequent underlying disorder (54.2%), but gastrointestinal symptoms are absent in 45.8% of patients. Pancreatic panniculitis has a predilection for the lower limbs, which are affected in 98.3% of cases. However, the cutaneous lesions may also involve the upper limbs and trunk. Arthritis is typically symmetric and polyarticular in nature, affecting both large and small joints. Of all patients who received treatment, 78.6% had a poor response. Death occurred in 27.1% of cases after a median duration of 8 (IQR 3.5-14) weeks.
Topics: Acute Disease; Adult; Arthritis; Female; Humans; Joints; Male; Middle Aged; Pancreatitis; Panniculitis
PubMed: 32776311
DOI: 10.1007/s10067-020-05333-8 -
The British Journal of Dermatology Apr 2016Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency (AATD) is a very rare disease. Its estimated prevalence is 1 in 1000 subjects with severe AATD... (Review)
Review
Neutrophilic panniculitis associated with alpha-1-antitrypsin deficiency (AATD) is a very rare disease. Its estimated prevalence is 1 in 1000 subjects with severe AATD (usually white individuals with a Pi*ZZ genotype). It is manifested clinically by painful recurrent ulcerating subcutaneous nodules, and characterized histologically by dense infiltrates of neutrophils in the deep dermis and connective-tissue septae, with secondary lobular panniculitis. It may be the only clinical manifestation of AATD, although it can also occur together with the classical pulmonary or hepatic manifestations of the disease. AATD-associated panniculitis is not only very rare but may also be significantly underdiagnosed. The physician managing a case of panniculitis with a clinical presentation suggestive of AATD and a compatible skin biopsy should measure serum AAT concentration and, if low, determine the AAT phenotype by isoelectric focusing. If uncertainty remains, the SERPINA1 gene should be sequenced to identify the genotype. If AATD is diagnosed, AATD testing of first-degree family members should be performed in order to take appropriate preventive and therapeutic measures, including genetic counselling, education on inheritance, risk arising from tobacco smoke, occupational exposure to pollutants and hepatotoxic substances, and the provision of information on clinical management. Cases of panniculitis in which conventional therapy with dapsone has failed may be managed with intravenous augmentative therapy using human AAT. The current manuscript addresses the fundamental concepts of the pathogenesis of AATD-associated panniculitis and describes the clinical presentation and management of cases in order to reduce underdiagnosis and improve outcomes.
Topics: Adolescent; Adult; Female; Humans; Male; Middle Aged; Neutrophils; Panniculitis; Young Adult; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
PubMed: 26595240
DOI: 10.1111/bjd.14309 -
American Journal of Transplantation :... Mar 2016Obesity is often associated with the development of adipose tissue (AT) inflammation, resulting in metabolic dysfunction and an increased risk for developing type 2... (Review)
Review
Obesity is often associated with the development of adipose tissue (AT) inflammation, resulting in metabolic dysfunction and an increased risk for developing type 2 diabetes. It is also associated with multiple chronic diseases, including cardiovascular, liver, and kidney disease, and thus can contribute to organ failure. Several studies have investigated whether there is a correlation between obesity and outcomes in transplantation, but there is currently very limited information on the specific role of AT inflammation in the rejection process or on the overall function of the transplanted organ. Here, we provide a brief review of the current understanding of the cellular mechanisms that control obesity-associated AT inflammation and summarize knowledge about how obesity affects clinical outcomes following solid organ or hematopoietic stem cell transplantation. We also highlight opportunities for more research to better understand how obesity affects outcomes of transplantation.
Topics: Adipose Tissue; Animals; Graft Rejection; Humans; Obesity; Organ Transplantation; Panniculitis
PubMed: 26755439
DOI: 10.1111/ajt.13578 -
JAMA Dermatology Oct 2022There is a knowledge gap about subcutaneous panniculitis-like T-cell lymphoma (SPTCL) owing to its rarity and diagnostic difficulty, resulting in an absence of...
Clinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.
IMPORTANCE
There is a knowledge gap about subcutaneous panniculitis-like T-cell lymphoma (SPTCL) owing to its rarity and diagnostic difficulty, resulting in an absence of well-documented large case series published to date.
OBJECTIVE
To generate consensus knowledge by a joint multi-institutional review of SPTCL and related conditions.
DESIGN, SETTING, AND PARTICIPANTS
This retrospective clinical and pathological review included cases initially diagnosed as SPTCL at 6 large US academic centers. All cases were reviewed by a group of pathologists, dermatologists, and oncologists with expertise in cutaneous lymphomas. Through a process of group consensus applying defined clinical and pathological diagnostic criteria, the cohort was classified as (1) SPTCL or (2) adipotropic lymphoproliferative disorder (ALPD) for similar cases with incomplete histopathological criteria for SPTCL designation.
EXPOSURES
Cases of SPTCL diagnosed between 1998 and 2018.
MAIN OUTCOMES AND MEASURES
The main outcome was disease presentation and evolution, including response to therapy, disease progression, and development of hemophagocytic lymphohistiocytosis.
RESULTS
The cohort of 95 patients (median [range] age, 38 [2-81] years; female-to-male ratio, 2.7) included 75 cases of SPTCL and 20 cases of ALPD. The clinical presentation was similar for both groups with multiple (61 of 72 [85%]) or single (11 of 72 [15%]) tender nodules mostly involving extremities, occasionally resulting in lipoatrophy. Hemophagocytic lymphohistiocytosis (HLH) was only observed in SPTCL cases. With a mean follow-up of 56 months, 60 of 90 patients (67%) achieved complete remission with a median (range) of 3 (1-7) cumulative therapies. Relapse was common. None of the patients died of disease progression or HLH. Two patients with ALPD eventually progressed to SPTCL without associated systemic symptoms or HLH.
CONCLUSIONS AND RELEVANCE
In this case series of patients initially diagnosed as having SPTCL, results showed no evidence of systemic tumoral progression beyond the adipose tissue. The SPTCL experience in this study confirmed an indolent course and favorable response to a variety of treatments ranging from immune modulation to chemotherapy followed by hematopoietic stem cell transplantation. Morbidity was primarily associated with HLH.
Topics: Humans; Male; Female; Adult; Lymphohistiocytosis, Hemophagocytic; Retrospective Studies; Neoplasm Recurrence, Local; Panniculitis; Lymphoma, T-Cell; Disease Progression
PubMed: 36001337
DOI: 10.1001/jamadermatol.2022.3347 -
The American Journal of Dermatopathology Mar 2018Panniculitides comprise a group of heterogeneous inflammatory diseases. Nevertheless, histopathological study along with clinicopathological correlation usually led to a... (Review)
Review
BACKGROUND
Panniculitides comprise a group of heterogeneous inflammatory diseases. Nevertheless, histopathological study along with clinicopathological correlation usually led to a specific diagnosis. In most textbooks, the first step in the diagnosis is to classify them as mostly septal or lobular depending on where the inflammatory infiltrate is located. The second step is deciding if vasculitis is present or not. Finally, the third step is further characterizing the inflammatory infiltrate. However, in addition to the algorithmic approach to panniculitis diagnosis, some subtle changes may help to the diagnosis.
OBJECTIVE
To review some clues in panniculitis dermatopathological diagnosis such as presence of granulation tissue, sclerotic connective tissue septa, small granulomas arranged around a central clear space, so-called ghost adipocytes, needle-shaped crystals, small lobules with a proliferation of capillaries, Splendore-Hoeppli phenomenon, refractile microspheres, neutrophilic infiltrates, granulomas and fibroplasia or presence of adipose tissue in dermis.
METHODS
We have compiled 12 clues based in our personal experience in this field.
LIMITATIONS
Specificity and sensibility of every clue may vary and these clues are a guide to correct diagnoses that should rely in clinicopathological correlation.
CONCLUSION
Knowledge of these 12 clues will help to increase the diagnostic accuracy in panniculitis diagnosis.
Topics: Humans; Panniculitis
PubMed: 29470303
DOI: 10.1097/DAD.0000000000000985 -
Journal of the European Academy of... Mar 2017Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by...
BACKGROUND
Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains.
OBJECTIVE
To depict the profile of children with recurrent idiopathic panniculitis.
METHODS
Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis.
RESULTS
Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed. In two patients, extensive involvement led to loss of the cutaneous fatty tissue. Laboratory abnormalities included increased acute phase reactants, leukocytosis with mild neutrophilia, microcytic anaemia and elevated liver enzymes. Histopathology showed lobar panniculitis without vasculitis and with a mixed infiltrate, composed of neutrophils, mononuclear cells, lymphocytes, macrophages and myeloid cells. Neutrophils and myeloid cells were more prominent in early lesions, whereas macrophages predominated in late stages, leading to lipophagia and lipoatrophy. Immunohistochemistry showed positive staining for myeloperoxidase around the necrotic adipocytes in early stages and CD68/PGM1 macrophages in late stages. Intense STAT1 staining was observed in the inflammatory infiltrate. All patients improved with methotrexate and corticosteroids.
CONCLUSION
We present five cases of lobar panniculitis and lipoatrophy in childhood. The clinico-pathologic presentation shares features with other autoinflammatory diseases.
Topics: Acute-Phase Proteins; Adipocytes; Adipose Tissue; Anemia; Antigens, CD; Antigens, Differentiation, Myelomonocytic; Atrophy; Child; Child, Preschool; Female; Humans; Infant; Leukocytosis; Lymphocytes; Macrophages; Male; Neutrophils; Panniculitis; Peroxidase; Recurrence; STAT1 Transcription Factor
PubMed: 27515780
DOI: 10.1111/jdv.13858 -
BMJ Case Reports Jan 2015
Topics: Aged, 80 and over; Carcinoma; Female; Humans; Pancreatic Neoplasms; Panniculitis; Paraneoplastic Syndromes
PubMed: 25631757
DOI: 10.1136/bcr-2014-206980 -
BMJ Case Reports Oct 2015
Topics: Abdominal Pain; Cholangiopancreatography, Magnetic Resonance; Female; Humans; Panniculitis; Retroperitoneal Space; Tomography, X-Ray Computed; Young Adult
PubMed: 26491006
DOI: 10.1136/bcr-2015-212670 -
Advances in Rheumatology (London,... Aug 2019The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous... (Review)
Review
The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.
Topics: Behcet Syndrome; Biopsy; Erythema Induratum; Erythema Nodosum; Etanercept; Humans; Immunosuppressive Agents; Infections; Panniculitis; Panniculitis, Lupus Erythematosus; Polyarteritis Nodosa; Rheumatologists; Subcutaneous Fat; Subcutaneous Tissue; Vasculitis
PubMed: 31370889
DOI: 10.1186/s42358-019-0077-5 -
Haematologica Oct 2023Germline HAVCR2 mutations are frequently detected in subcutaneous panniculitis-like T-cell lymphoma (SPTCL) patients with/without hemophagocytic lymphohistiocytosis... (Meta-Analysis)
Meta-Analysis
Germline mutations and their relation to the clinical spectrum of subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis: results from a multicenter study and meta-analysis.
Germline HAVCR2 mutations are frequently detected in subcutaneous panniculitis-like T-cell lymphoma (SPTCL) patients with/without hemophagocytic lymphohistiocytosis (HLH) but factors associated with variable manifestations remain undetermined. To evaluate clinical variations and associated factors in SPTCL and/or HLH with/without HAVCR2 mutations, we performed direct sequencing of HAVCR2 exon 2 using DNA from patients with SPTCL or idiopathic HLH/HLH-like systemic illnesses, defined by HLH alone without secondary causes. The systematic review and individual patient data (IPD) level meta-analysis which included the present and previously published studies reporting HAVCR2 mutations in SPTCL with/without HLH populations was subsequently conducted using random-effects meta-analysis and multivariate logistic regression. Among 34 patients enrolled, ten of 28 SPTCL patients developed HLH/HLH-like systemic illnesses. Six cases with HAVCR2Y82C mutation manifested with HLH without panniculitis. Male sex (P=0.03) and age <18 years (P=0.04) were associated with HLH, corresponding to the inverse correlation between age and HLH-2004 score (r=-0.40; P=0.02). Homozygous HAVCR2Y82C mutation was more common in the presence of HLH compared with the absence (75.0% vs. 44.4%; P=0.02). Using IPD from the present and the other three eligible cohorts (N=127), male sex, heterozygous and homozygous/compound heterozygous HAVCR2 mutations were associated with HLH by the adjusted odds ratio of 2.93 (95% confidence interval [CI]: 1.22-7.06), 4.77 (95% CI: 1.05-21.63) and 8.48 (95% CI: 2.98-24.10), respectively. Patients with male sex and/or germline HAVCR2 mutations showed an increased risk of developing HLH. Younger patients tended to manifest with HLH, while older patients typically presented with SPTCL with less frequent HLH/HLH-like systemic illnesses.
Topics: Humans; Male; Adolescent; Lymphohistiocytosis, Hemophagocytic; Panniculitis; Germ-Line Mutation; Germ Cells; Hepatitis A Virus Cellular Receptor 2; Multicenter Studies as Topic
PubMed: 37051767
DOI: 10.3324/haematol.2022.282419