Natural history of spontaneous pancreatic portal vein fistulae: A systematic review of the literature.

BACKGROUND: Spontaneous pancreatic portal vein fistula (PPVF) - a rare complication of pancreatic inflammation - varies widely in presentation and means of diagnosis but has been previously associated with bleeding complications and mortality. A systematic review of published literature was performed to assess the frequency of outcomes. METHODS: A search of electronic databases (PubMed, Ovid MEDLINE, Scopus, EMBASE, gray literature) resulted in 1667 relevant unique manuscripts; 52 met inclusion criteria. RESULTS: A total of 74 unique (male n = 47, 63.5 %) patients were included. Mean age was 53.5 (±11.9) years. History of alcohol use was reported in 55 (74.3 %). Underlying chronic pancreatitis (CP) was present in 49 (66.2 %). In cases where presenting symptoms were reported (n = 57, 77.4 %), the most frequent were abdominal pain (63.5 %), weight loss (14.9 %), rash (12.2 %), nausea/vomiting (12.2 %), and polyarthritis (9.5 %). Computed tomography was the most common imaging modality used to confirm the diagnosis (n = 20, 27.0 %), followed by magnetic resonance cholangiopancreatography (n = 14, 18.9 %). Portal vein thrombosis was reported in 57 (77.0 %), and bleeding events (luminal, variceal, or intra-pseudocyst) were reported in 13(17.6 %) patients. Younger age was associated with higher risk of bleeding events. Mortality was reported in 12 (16.2 %) patients at any time during follow up. Older age and polyarthritis at presentation were associated with mortality. CONCLUSIONS: PPVF is a rare and potentially fatal condition, though rates of bleeding complication and death were relatively low in this population. High-quality observational studies are needed to better understand the pathophysiology and natural history of this diagnosis.

Treatment outcomes between cyclosporin and chemotherapy in adult subcutaneous panniculitis-like T-cell lymphoma: a report from nation-wide Thai lymphoma study group registry.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of T-cell lymphomas with a characteristic feature of subcutaneous nodules associated with hemophagocytic lymphohistiocytosis (HLH). Treatment options for SPTCL are mainly chemotherapy (CMT) or immunosuppressive agents with selection currently dependent on physician decisions. Outcomes between the 2 treatment remedies have not yet been comprehensively compared. This study aimed to compare complete remission (CR) rates between SPTCL patients receiving cyclosporin (CSA)-based regimen (CSA +/- steroid) and CMT. The 5-year overall survival (OS) and 5-year progression free survival (PFS) were also analyzed. Clinical data from patients with SPTCL were drawn from the Thai Lymphoma Study Group registry who were newly diagnosed between 2007 and 2023. A total of 93 patients were selected with 45 cases having received CSA-based regimen and 48 cases having received CMT. There were more patients with limited stage at skin in the CSA group (63.8% vs. 36.2%, p = 0.003), while more patients with hepato- and/or splenomegaly were found in the CMT group (56.2% vs. 24.5%; p = 0.002). Germline HAVCR2 mutations were detected in 26/33 (78.8%) cases. The CR rate was significantly higher in patients treated with CSA (87% vs. 58.3%; OR = 6.5 [95%CI, 2.7-15.3]; p = 0.002). At a median follow-up of 87.8 months (range 0-185), the 5-year OS (98% vs. 87%, p = 0.19) and PFS (72.4% vs. 69.2%, p = 0.19) showed a trend favoring patients treated with CSA. Based on our study, CSA-based regimens are the preferred first-line treatment remedy for newly diagnosed SPTCL, especially in patients with limited cutaneous involvement.

Hemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors.

Hemophagocytic lymphohistiocytosis (HLH) has been described for decades in association with malignancies (M-HLH). While its mechanism is unknown, M-HLH has a poor prognosis, ranging from 10% to 30% overall survival. Mature T-cell lymphomas, diffuse large B-cell lymphoma, and Hodgkin lymphoma, with or without viral co-triggers such as Epstein-Barr virus, are among the most frequent underlying entities. Most M-HLH cases occur at the presentation of malignancy, but they may also occur during therapy as a result of immune compromise from chemotherapy (HLH in the context of immune compromise, IC-HLH) and (typically) disordered response to infection or after immune-activating therapies (Rx-HLH, also known as cytokine release syndrome, CRS). IC-HLH typically occurs months after diagnosis in the context of fungal, bacterial, or viral infection, though it may occur without an apparent trigger. Rx-HLH can be associated with checkpoint blockade, chimeric antigen receptor T-cell therapy, or bispecific T-cell engaging therapy. Until recently, M-HLH diagnosis and treatment strategies were extrapolated from familial HLH (F-HLH), though optimized diagnostic and therapeutic treatment strategies are emerging.

Clinical analysis of 10 cases with subcutaneous panniculitis-like T-cell lymphoma and tissue AURKA expression.

BACKGROUND: Due to its rarity, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is often misdiagnosed as benign panniculitis, and there are no standardized treatment guidelines for SPTCL. Aurora kinase A (AURKA) plays a regulatory role in both mitosis and meiosis. Cells treated with an AURKA inhibitor showed severe mitotic delay, which triggered apoptosis. MATERIALS AND METHODS: Ten cases of SPTCL were collected in this study, and immunohistochemistry was performed to detect AURKA expression in the skin tissues of these cases. Control groups were set as follows: 1) 10 cases of inflammatory panniculitis; 2) 9 healthy individuals. Fisher's exact test was used to compare the positive rates of AURKA among various groups. RESULTS: An average onset age of 27.3 years was found in 10 SPTCL cases. Clinically, these patients primarily presented with multiple subcutaneous nodules on the trunk and lower extremities, accompanied by intermittent high fever. One case showed lymph node metastasis, while no other distant organ metastasis being observed in any case. Pathologically, there was an infiltration of a large number of atypical lymphocytes within the fat lobules, characterized as a cytotoxic type. AURKA stanning was positive in 6 out of 10 SPTCL cases, while no positive cases were found in the control groups. CONCLUSION: 1) SPTCL predominantly affects young individuals and can be identified by nodular erythema on the trunk, intermittent high fever, and infiltration of atypical cytotoxic lymphocytes within fat lobules. 2) For early-stage cases without metastasis, monotherapy with glucocorticoids or immunosuppressants such as cyclosporine can be considered. 3) High expression of AURKA in SPTCL tissues suggests that AURKA could be a potential biomarker for disease diagnosis, providing a theoretical basis for further targeted therapy.

Mesenteric Panniculitis.

Background: Mesenteric panniculitis (MP) is an uncommon non-neoplastic idiopathic inflammation of adipose tissue, mainly affecting the mesentery of the small intestine, with its etiology remaining largely speculative. The difference in prevalence of MP among females and males varies across multiple studies. In most cases, MP is asymptomatic; however, patients can present with nonspecific abdominal symptoms or can mimic underlying gastrointestinal and abdominal diseases. The diagnosis is suggested by computed tomography and is usually confirmed by surgical biopsies if necessary. Treatment is generally supportive and based on a few selected drugs, namely, nonsteroidal anti-inflammatory drugs or corticosteroids. Surgery is reserved when the diagnosis is unclear, when malignancy is suspected or in the case of severe presentation such as mass effect, bowel obstruction, or ischemic changes. Summary: MP is a rare inflammatory condition of the mesentery often asymptomatic but can cause nonspecific abdominal symptoms. Diagnosis relies on computed tomography imaging, with treatment mainly supportive, utilizing medications like nonsteroidal anti-inflammatory drugs or corticosteroids, while surgery is reserved for severe cases or diagnostic uncertainty. Key Messages: MP causes abdominal pain, and it is mainly diagnosed with CT scan.

Different mutational profiles of subcutaneous panniculitis-like T-cell lymphoma and lupus panniculitis: an additional case series.

BACKGROUND AND OBJECTIVE: subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma with indolent behavior, mostly present in women and associated with immunological diseases whose pathogenic background is still poorly understood. SPTCL is associated with lupus erythematosus panniculitis (LEP) and histologically misdiagnosed. OBJECTIVES: the aim of our study was to identify mutations affecting the pathogenesis of both SPTCL and LEP. MATERIALS AND METHODS: we studied a total of 10 SPTCL and 10 LEP patients using targeted Next Generation Sequencing and pyrosequencing. Differences in gene expression between molecular subgroups were investigated using NanoString technology. Clinical data were collected, and correlations sought with the molecular data obtained. RESULTS: the mutational profile of SPTCL and LEP is different. We identified fewer pathogenic mutations than previously reported in SPTCL, noting a single HAVCR2-mutated SPTCL case. Interestingly, 40% of our SPTCL cases showed the pathogenic TP53 (p.Pro72Arg) (P72R) variant. Although cases showing HAVCR2 mutations or the TP53 (P72R) variant had more severe symptomatic disease, none developed hemophagocytic syndrome (HPS). Furthermore, TP53 (P72R)-positive cases were characterized by a lower metabolic signaling pathway and higher levels of CD28 expression and Treg signaling genes. In addition, 30% of our cases featured the same mutation (T735C) of the epigenetic modificatory gene DNMT3A. None of the LEP cases showed mutations in any of the studied genes. CONCLUSIONS: the mutational landscape of SPTCL is broader than previously anticipated. We describe, for the first time, the involvement of the TP53 (P72R) pathogenic variant in this subgroup of tumors, consider the possible role of different genetic backgrounds in the development of SPTCL, and conclude that LEP does not follow the same pathogenic pathway as SPTCL.

A double-blind pilot study of oral baricitinib in adult patients with lupus erythematosus panniculitis.

Lupus erythematosus panniculitis (LEP) is a chronic inflammatory skin disease with a significant impact on the overall well-being of patients. The safety and efficacy of oral baricitinib for the treatment of LEP have not been studied. This study aimed to explore the efficacy of oral baricitinib in patients with LEP who are recalcitrant or intolerant to conventional therapies. Patients (aged ≥18 years) with active LEP (with a revised cutaneous lupus erythematosus disease area and severity index [RCLASI]-active score ≥4] were randomly assigned 2:1 to baricitinib (4 mg) or placebo (once daily for 20 weeks). The placebo group was switched to baricitinib (4 mg) at week 13, and the final evaluation was conducted at week 24. The primary endpoint was the proportion of patients with an RCLASI-A score decreased by 20% at week 12. The secondary endpoints included the changes in the Cutaneous Lupus Erythematosus Disease Area and Severity Index active-(CLASI-A) score, the Dermatology Life Quality Index (DLQI), the Physician's Global Assessment (PGA) score, and safety. Five patients were enrolled. Three patients received baricitinib (4 mg), and two patients were treated with placebo. Two patients in the baricitinib treatment group showed a significant RCLASI-A decrease at week 12 and week 24. Two patients in the placebo group had no change in RCLASI-A at week 12 and a significant decrease at week 24. No new safety events were observed. Treatment with baricitinib was effective and well tolerated in patients with LEP.

Association between mesenteric panniculitis and urolithiasis.

BACKGROUND: Mesenteric panniculitis is a rare condition and refers to benign and nonspecific inflammation of mesenteric fat. OBJECTIVES: This study aimed to evaluate the hypothesis of a greater prevalence of mesenteric panniculitis in patients with urolithiasis. MATERIALS AND METHODS: In this cross-sectional study, abdominopelvic CT scans of 500 patients were reviewed for the presence of urolithiasis and mesenteric panniculitis. The inclusion criteria were patients who were referred with acute abdominal pain and were suspected of having urolithiasis or other urinary conditions and who had undergone abdominopelvic CT scan. Subcutaneous fat thickness was measured, and pain intensity was recorded by patient evaluation. RESULTS: Mesenteric panniculitis was found in 10 patients, all of whom (100%) had urinary stones (ureter or kidney or both), and none of them had previous surgeries or known malignancies. The prevalence of panniculitis was significantly greater in the group with urolithiasis. In the urolithiasis group, subcutaneous fat thickness was greater in patients with panniculitis, although the difference was not statistically significant. In the subgroup analysis, pain intensity was not significantly greater in patients with panniculitis. CONCLUSION: Mesenteric panniculitis is more prevalent among patients with urolithiasis, but it seems that it does not change the intensity of the pain.

Severe Bilateral Sialadenitis of the Mandibular and Parotid Salivary Glands with Severe Panniculitis in a 2-Year-old Standard Poodle.

A 2-year-old male neutered Standard Poodle weighing 17.9 kg was presented to their primary care veterinarian for enlarged bilateral submandibular swellings following an interdog altercation sustained in the previous weeks. Cytology performed following fine-needle aspirates of the regions of swelling was inconclusive, and the patient was treated empirically with Clavaseptin. Despite treatment, the submandibular swellings continued to enlarge, and right-sided intermittent epistaxis was reported. On biochemical profile, there was mild hypercalcemia and mild hyperglobulinemia. The computed tomography (CT) findings were indicative of severe multifocal sialadenitis with severe regional cellulitis and inflammatory lymphadenopathy. Histopathology and cytology results described mixed inflammation of the salivary gland. Methenamine silver staining and Fite's acid-fast staining were negative. Aerobic and anaerobic cultures were negative. Targeted, next-generation DNA sequencing detected no known fungi or bacterial pathogens. These findings were consistent with the diagnosis of severe bilateral mandibular sialadenitis, panniculitis, and lymphadenopathy. The patient was prescribed enrofloxacin, clindamycin, phenobarbital, and prednisolone for 1 month. One week after initiating treatment, the patient had a significant reduction in size of the salivary glands. CT imaging was helpful in the diagnosis of this patient and allowed the clinician to identify which submandibular anatomical structures were abnormal, guiding subsequent diagnostic decisions to provide medical management to resolve the condition.

Linear and Annular Lupus Panniculitis of the Scalp with Annular Lupus Panniculitis of the Face in a Child.

Linear and annular lupus panniculitis of the scalp is a rare form of lupus panniculitis recently reported in literature. It presents as linear or annular or arciform areas of nonscarring alopecia of the scalp with minimal surface changes. We report a 4-year-old Indian female child with arciform erythematous plaque over the forehead extending on the scalp with nonscarring alopecia with annular erythematous plaque over the cheek. Histology showed lobular lymphocytic panniculitis with abundant mucin deposition. Antinuclear antibody and systemic screen for lupus were negative. After treatment with oral corticosteroids, complete remission was achieved with good regrowth of scalp hair with no relapse during the follow-up period of 6 months. This is the youngest reported case of linear and annular lupus panniculitis of the scalp.

Generalized Telangiectasia as a Hallmark of Intravascular Lymphoma: A Case Report and Literature Review.

A 55-year-old woman had remarkably generalized telangiectasia. Large atypical lymphoid cells were found within the lumen of small vessels, and CD20 was positive in her third skin biopsy. She was diagnosed with intravascular large B-cell lymphoma (IVLBCL) and went into remission of the skin manifestations after seven courses of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and four courses of intravenous high-dose methotrexate (HD MTX). To our knowledge, this is the first case report of IVLBCL with generalized telangiectasia in China. From a review of the literature, we found that generalized telangiectasia is a remarkable manifestation of intravascular lymphoma, and the differential diagnosis of intravascular lymphoma and panniculitis or vasculitis is very important. When the diagnosis is confusing, multiple skin biopsies are useful.

Pancreatitis, Panniculitis, and Polyarthritis Syndrome in Two Patients: A Case Series and Literature Review.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome presents a unique challenge in diagnosis and management because of its rarity and heterogeneous initial presentation. This manuscript presents a case series of two patients with PPP syndrome, shedding light on the diagnostic process and care for this uncommon condition. PPP syndrome is characterized by the simultaneous occurrence of pancreatitis or pseudocysts alongside polyarthritis and panniculitis. While its exact pathophysiology remains obscure, pancreatic inflammation is assumed to trigger the hematogenous dissemination of pancreatic enzymes, leading to fat necrosis and subsequent panniculitis, as well as chondronecrosis and/or osteonecrosis causing polyarthritis. Despite its recognition in medical literature since the late 1980s, PPP syndrome remains poorly understood, with only a limited number of cases reported globally. Its rarity and varied initial manifestations often result in misdiagnosis, causing delays in appropriate treatment. The presented case series highlights key clinical features and diagnostic clues of PPP syndrome. Both patients exhibited initial symptoms of inflammatory polyarthritis, accompanied by characteristic findings of "ghost cells" on skin biopsy. Additionally, radiographic and laboratory evidence revealed pancreatic changes consistent with this syndrome. This case series underscores the importance of multidisciplinary collaboration in managing PPP syndrome. Early recognition and accurate diagnosis are pivotal in initiating prompt and effective therapeutic interventions, thereby improving patient outcomes and minimizing long-term sequelae.

Clinicopathological Characteristics and Outcome of Cytophagic Histiocytic Panniculitis: A Single-Center, Retrospective Study.

Background: Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, mimicking hemophagocytic lymphohistiocytosis (HLH) and subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Purpose: To establish the clinicopathological features and response to treatment of CHP and evaluate the prognosis of patients and guide therapy based on the current state of knowledge. Material and Methods: Clinical, laboratory, histopathological, and outcome data of 12 patients with CHP were retrospectively collected between 2009 and 2022. Results: All the patients presented with plaques or nodules, mostly located in the lower extremities (11/12). Fewer cases involved systemic symptoms (9/12) and laboratory abnormalities (6/12), and none were positive for serum Epstein-Barr virus (EBV)-DNA. Histopathological examination revealed mixed septal and lobular inflammatory infiltration of histiocytes and lymphocytes. Large or atypical lymphocytes were rarely present (2/12). In some patients, varying proportions of plasma cells, neutrophils, and eosinophils were observed. The extent of histocytophagy was mild (9/12), moderate (2/12), and severe (1/12). HLH was not observed in any of our cases, none of which were fatal. Conclusion: The uniqueness of our study lies in the presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders (CTD) and streptococcal infections. Our study reveals that EBV-negative CHP tends to a better prognosis than previously research, filling the gap in the much-needed details of CHP in the Chinese population. Moreover, CHP may present as a reactive process in combined primary diseases; further studies are required to validate these findings.

Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, also may be present in hemophagocytic lymphohistiocytosis and subcutaneous panniculitis-like T-cell lymphoma. The presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders and streptococcal infections. In addition, EBV-negative CHP has a better prognosis than previously thought and provides knowledge of its prognosis in the Chinese population. With changes in the disease pedigree supported by the development of medical technology, CHP may present as a reactive process of a combined primary disease.

[Small bowel obstruction with an unusual background]. / Egy szokatlan hátteru vékonybél ileus.

Bevezetés: Kompressziós vékonybél ileus esetét ismertetjük, amelyet a bélfodor nem gyakori, inflammatorikus természetu betegsége, mesenterialis panniculitis idézett elo. A magyar szakirodalomban ilyen közléssel nem találkoztunk. Esetismertetés: A 91 éves férfi akut hasi panaszokkal került kórházba. A vizsgálatokkal vékonybél ileus derült ki. Ennek hátterében mutétkor malignitásra gyanús, bélfodri multinodularis elváltozást fedtünk föl. A biopsziából mesenterialis panniculitist diagnosztizáltunk. A ritka, több nyitott kérdéssel terhelt entitást mutatjuk be az irodalom és a saját észleleteink tükrében. Következtetések: Számos differenciáldiagnosztikai eshetoség figyelembevételével a kórkép szövettanilag igazolható. A diagnózis felállítása után a további teendoket az egyéb leletek és az adott klinikai kontextus gondos elemzése fogja meghatározni.

[Erythema nodosum]. / Erythema nodosum.

Erythema nodosum (EN) is the most frequently occurring form of acute panniculitis. It is characterized by painful red to livid raised nodules or bumps that typically occur symmetrically in the shin area. The cause of EN is often a reaction of the immune system to various triggers including infections, inflammatory diseases or medications. In approximately half of the cases no trigger can be identified. After treatment of the underlying pathology EN is typically self-limiting.

An unusual case of acquired generalized lipodystrophy (panniculitis variety).

Acquired generalized lipodystrophy (AGL) is a rare disease characterized by variable loss of adipose tissue and concurrent metabolic derangements, typically with childhood or adolescent onset. AGL has three subclassifications: panniculitis (type 1), autoimmune disease (type 2), and idiopathic (type 3). This report highlights a rare case of AGL type 1 in a previously healthy 3-year-old female who presented with diffuse erythematous subcutaneous nodules, progressive lipoatrophy, and histopathological findings of a lobular panniculitis.

Subcutaneous fat sclerosis in a domestic short-hair cat.

The authors describe a case of presumptive feline subcutaneous fat sclerosis, a condition reported only once previously (in 1987) and diagnosed with postmortem examination. We describe radiographic, computed tomography scan and histopathological findings, and partial response to oral methylprednisolone and Vitamin E.

A Case of Erythema Nodosum in a 20-Year-Old Female During the Postpartum Period.

Erythema nodosum (EN) is the most common form of panniculitis and occurs in about one in 100,000 people. EN typically presents as an eruption of tender, erythematous nodules on the anterior aspect of the legs, although the face, trunk, and arms can also be involved. While the majority of cases are idiopathic, a subset of cases occurs in association with various triggers, including infections, medications, tumors, and autoimmune diseases. Rarely can EN develop in relation to pregnancy, which is thought to provide a physiologic background that favors its development. While pregnancy has been associated with EN in a minority of cases, currently, there is a limited amount of data suggesting that EN can develop in the late postpartum period. Herein, we present a case of a 20-year-old female with a six-week history of painful lesions on her lower extremities. A physical exam revealed multiple tender, erythematous nodules on the anterior aspect of the lower extremities, spanning from the knees to the toes. Laboratory workup showed no other identified triggers of EN in our patient besides pregnancy. Management of EN in our patient involved a low dose, six-day course of prednisone (initial dose of 15 mg/day) and ibuprofen for one week, leading to symptomatic improvement. Our case emphasizes the possibility of EN presenting in the late postpartum period. This case underscores the importance of considering EN in the differential diagnoses for women presenting with compatible lesions postpartum.