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.

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.

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.

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.

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.

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.

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.

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.

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.

[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.

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.

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.

Mesenteric panniculitis associated with non-alcoholic fatty liver disease: A case series.

Mesenter ic p anniculitis (MP) is a b enign infla mmatory condi tion of the abdomin al mesentery, whi ch presents with a wid e variety of symptoms. I t is diagnosed non - invasively through com puted to mography (CT ) scan, whereas biopsy is still co nside red th e gold standa rd. Steroids are the first line of treatment. Here, we report four cases who presented with abdominal pain. These patients were overweight and the CT scan findings were suggestive of mese nte ric panniculitis. Three cases had concomitant non- alcoholic steatohep atitis w ith el evated alanine transaminase levels, dyslipidaemia, and insulin resistance. FibroSca n showed moderate to severe steatosis. PNPLA3 rs738409 genotype was homozygous positive (GG) in one patient, whereas two patients were heterozygous positive (CG ). This a ssociat io n has not been well-described so far and w arrants f ur ther inve s tigation. There may be some common predisposing factors.

Fat Stranding Associated With High-Grade Colon Adenocarcinoma: A Case Report.

Abdominal pain is a common complaint among patients who present to the emergency department. In this setting, a CT scan of the abdomen is frequently used for diagnostic purposes. Fat stranding is an important and relevant CT finding. It is non-specific and can be associated with multiple conditions that range from benign to life-threatening. Although it may not provide the final diagnosis, it can direct the evaluating physician toward an area of concern. This case report describes an 81-year-old female presenting to the emergency department with diffuse abdominal pain. CT of the abdomen/pelvis showed mesenteric fat stranding. She was eventually diagnosed with high-grade adenocarcinoma of the colon. The radiological appearance, pathophysiology, possible etiologies, and clinical significance of fat stranding are discussed.

A rare case of polyarthritis panniculitis and pancreatitis.

Extra pancreatic manifestations of pancreatitis are rare, with a prevalence of 2-3%. One such rare manifestation is the triad of joint pain (polyarthritis), tender skin lesions (panniculitis), and pancreatic inflammation (pancreatitis), known as PPP. The pathogenesis of this phenomenon is not fully understood but is believed to involve lipolysis by pancreatic enzymes at lipid-rich skin and joint sites. PPP primarily affects middle-aged males with a history of alcohol use disorder. Diagnosis can be challenging due to the absence of typical abdominal symptoms. Delayed diagnosis may significantly worsen outcomes. Supportive therapy is the mainstay, but resolution requires addressing the underlying pancreatic abnormality. We present a case of a patient with a history of alcohol use disorder and recurrent acute pancreatitis who developed joint pain and skin rash. Extensive work-up ruled out other causes, and imaging and biopsy confirmed the diagnosis of PPP. Symptomatic management and treatment of the underlying pancreatic abnormality led to complete resolution of symptoms. Our case serves to raise awareness of this rare but potentially fatal syndrome.