Subcutaneous panniculitis-like T-cell lymphoma: fever and facial swelling with hemophagocytic syndrome.

We report a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a young man presenting with fever and facial swelling. He had pancytopenia and hemophagocytic syndrome (HPS) on evaluation. The histopathological examination of skin punch biopsy from the face and chest wall showed SPTCL. Given the associated HPS, he was started on steroid and multidrug chemotherapy following which he had symptomatic improvement.

Neutrophilic Panniculitides.

Neutrophilic panniculitides are a heterogeneous group of inflammatory disorders encompassing many different entities. This review article focuses on the epidemiology, pathogenesis, clinicopathological features, diagnosis, and treatment of selected diseases. Patients often seek care due to systemic involvement, but the variable presentation of panniculitides can present a diagnostic challenge. Most therapeutic modalities for neutrophilic disorders are anecdotal at best with a notable lack of standardization of the responses to medications. There is an urgent need for a larger multi-institutional collaboration to address the unmet needs of these challenging, yet rare conditions.

Serial lipase measurements in pancreatic panniculitis.

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.

Pancreatitis, panniculitis and polyarthritis (PPP) syndrome.

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.

Panniculitis with late onset enthesitis-related arthritis: a case report.

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.

[Characteristics of the course of lupus erythematosus panniculitis in a retrospective analysis of 17 patients]. / A lupus erythematosus panniculitis lefolyásának jellegzetességei 17 betegünk retrospektív vizsgálata alapján.

INTRODUCTION: Lupus erythematous panniculitis (LEP) is a rare type of chronic cutaneous lupus erythematous. Clinical characteristics are tender, subcutaneous nodules, erythematous plaques. Disfigurement of face and body might develop which affects the patient's quality of life. LEP can be the first sign of systemic lupus erythematous (SLE). OBJECTIVE: Our aim was to review the clinicopathological characteristics and the course of LEP through our own patients. METHODS: We retrospectively analyzed the clinical records of 17 LEP patients at Semmelweis University's Department of Dermatology, Venerology and Dermatooncology between 2000 and 2022. RESULTS: The male : female ratio was 1 : 16, average age was 37.8 years. Lesion localisations were proximal lower (8/17) and upper extremities (7/17), face (4/17), breast (3/17), chest (2/17), buttocks (2/17), back (1/17) and distal lower extremity (1/17). Lesion morphologies were nodules (11/17), plaques (7/17), lipoatrophy (4/17), ulceration (3/17), calcification (1/17). Discoid changes covered in 6 cases. In 10 cases, systemic symptoms were observed (arthritis (4/17), haematological (5/17), renal (2/17), anti-phospholipid syndrome (2/17). 7 patients fulfilled the EULAR/ACR criteria for SLE. Histology showed mixed type panniculitis in 8, lobular in 3 cases. Average time until diagnosis was 24.3 months. Among all our SLE patients, skin symptoms regressed following systemic immunosuppressive treatment. LEP patients with only skin manifestation were often resistant for the therapy of cutaneous lupus erythematous. CONCLUSION: The diagnosis of LEP often takes months or years. Wider knowledge of LEP would shorten the time to diagnosis, preventing disfigurement and possible damage of internal organs. Based on our observations, LEP without SLE might be treated with early immunosuppression. Orv Hetil. 2023; 164(5): 172-178.

Orbital Mass as the Only Presenting Sign with Overlapping Features of Lupus Erythematosus Panniculitis and Subcutaneous Panniculitis-Like T-Cell Lymphoma.

PURPOSE: Even though subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus panniculitis (LEP) are two separate entities, recently they were claimed to represent two ends of a spectrum of T-cell-mediated orbital lymphoproliferative diseases. METHODS: A 78-year-old woman presented with a 1-month history of right-sided periorbital swelling and redness. There was a palpable mass in the medial right lower eyelid with restriction of upward and lateral gaze. MRI revealed a 14 × 7 mm hypointense lesion in the anteromedial orbit. RESULTS: The systemic and ocular findings, orbital biopsy with histopathology and immunochemistry showed overlapping features of LEP and SPTCL. The patient was consulted with rheumatology and hematology, and the physicians arrived at a consensus that the patient existed in the above-mentioned disease spectrum. She was started on systemic immunosuppressive treatment and her clinical findings improved substantially. CONCLUSIONS: This is the first report of a patient, who presented with orbital mass causing vision loss and gaze restriction that had overlapping clinical and histopathologic features of LEP and SPTCL consistent with this novel disease spectrum, in the literature.

Self-induced panniculitis in adolescence: Three cases and review of the literature.

Self-induced trauma is a rare cause of panniculitis in adolescents and may be associated with undiagnosed underlying psychological conditions. The condition often poses a diagnostic challenge as patients usually present with non-specific signs and symptoms, and often a concealed history of self-induced trauma. Here, we present three adolescent patients with self-induced traumatic panniculitis. All three patients were repeatedly admitted with non-specific cutaneous lesions as well as multiple somatic complaints. After extensive evaluation to exclude organic causes, psychiatric assessment eventually revealed underlying psychosomatic disorders as a cause of the self-induced trauma.

Visceral extra-abdominal panniculitis after COVID19.

"Retractile mesenteritis" was the first name given to a rare, benign, inflammatory disease that affects the adipose tissue of the intestinal mesentery and less frequently other locations. Now labeled as mesenteric panniculitis, the pathogenic mechanism remains unclear. Several stimuli could be involved, and it is sometimes associated with other conditions such as malignancy or autoimmune diseases. We present a case of mesenteric panniculitis with extensive abdominal and extra-abdominal involvement that developed a few months after SARS-COV2 infection, raising the hypothesis of this virus as a potential trigger for autoinflammatory and autoimmune diseases.

Painful subcutaneous nodules in an alcoholic: a case of pancreatic panniculitis.

Pancreatic panniculitis is an uncommon manifestation of pancreatic disease, affecting approximately 2-3% of patients with acute or chronic pancreatitis [1]. Its accurate diagnosis is crucial for effective treatment as skin manifestations usually precede systemic symptoms of pancreatitis. The diagnosis of pancreatic panniculitis requires identifying and confirming pancreatic disease in addition to the typical histopathological findings of lobular panniculitis without vasculitis. Herein, we present a patient with pancreatic panniculitis, underlying acute pancreatic failure, and kidney injury.

Germline BRCA2 variants in advanced pancreatic acinar cell carcinoma: A case report and review of literature.

BACKGROUND: Pancreatic acinar cell carcinoma (PACC) is a rare tumor. Up to 45% of PACCs have alterations in the DNA damage repair pathway and 23% harbor rearrangements in the BRAF or RAF1 genes. We present a PACC case with a germline BRCA2 likely pathogenic variant (LPV) to highlight the impact of genomic testing on treatment decisions and patient outcomes. In our larger case series, we provide clinic-based information on additional 10 PACC patients treated in our center. CASE SUMMARY: A 70-year-old male was diagnosed with advanced PACC. At presentation, he was cachectic with severe arthralgia despite prednisolone and a skin rash that was later confirmed to be panniculitis. He was treated with modified FOLFIRINOX (mFFX) with the knowledge of the germline BRCA2 LPV. Following 11 cycles of mFFX, a computed tomography (CT) scan demonstrated significant tumor response in the pancreatic primary and hepatic metastases, totaling 70% from baseline as per Response Evaluation Criteria in Solid Tumors. Resolution of the skin panniculitis was also noted. We identified two additional PACCs with druggable targets in our case series. Our data contribute to practical evidence for the value of germline and somatic profiling in the management of rare diseases like PACC. CONCLUSION: This patient and others in our larger case series highlight the importance of genomic testing in PACC with potential utility in personalized treatment.

[Ruxolitinib as an effective treatment for panniculitis associated hemophagocytic syndrome: A report of 2 cases and literature review].

Hemophagocytic syndrome (HPS) is a severe disease characterized by excessive release of inflammatory cytokines caused by abnormal activation of lymphocytes and macrophages, which can cause multiple organ damage and even death. Panniculitis is a disease characterized by inflammation of subcutaneous adipose tissue. We effectively treated 2 patients with panniculitis-associated HPS with ruxolitinib. Case 1: A 70-year-old male started with intermittent plantar swelling and pain, and then developed leukocytosis, mild anemia, multiple red maculopapules with painless subcutaneous nodules on the forehead, neck and bilateral lower legs. The patient was treated with prednisone and leflunomide for improvement. After that, repeated fever and rash occurred again. After admission to our hospital, we found his leukocyte and hemoglobin decreased, ferritin raised, fibrinogen and natural killer (NK) cell activity decreased, and hemophagocytic cells were found in bone marrow aspiration. The skin pathology was consistent with non-suppurative nodular panniculitis. He was diagnosed with nodular panniculitis associa-ted HPS. He was treated with glucocorticoid, cyclosporine, etoposide and gamma globule, but the disease was not completely controlled. After adjusting etoposide to ruxolitinib, his symptoms and abnormal laboratory findings returned to normal. After 2 months he stopped using ruxolitinib due to repeated infections. During the follow-up, though the prednisone dose was tapered, his condition was stable. Case 2: A 46-year-old female patient developed from intermittent fever, erythematous nodular rash with tenderness, leukopenia, and abnormal liver function. antibiotic therapy was ineffective. She improved after glucocorticoid treatment, and relapsed after glucocorticoid reduction. There were fever, limb nodules, erythema with ulcerative necrosis, intermittent abdominal pain when she came to our hospital. Blood examination showed that her white blood cells, red blood cells and platelets were decreased, fibrinogen was decreased, triglyceride was increased, ferritin and soluble interleukin-2 receptor(SIL-2R/sCD25) were significantly raised, and hemophagocytic cells were found in bone marrow aspiration. It was found that Epstein-Barr virus DNA was transiently positive, skin Staphylococcus aureus infection, and pulmonary Aspergillus flavus infection, but C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were normal, and no evidence of tumor and other infection was found. Skin pathology was considered panniculitis. The diagnosis was panniculitis, HPS and complicated infection. Antibiotic therapy and symptomatic blood transfusion were given first, but the disease was not controlled. Later, dexamethasone was given, and the condition improved, but the disease recurred after reducing the dose of dexamethasone. Due to the combination of multiple infections, the application of etoposide had a high risk of infection spread. Ruxolitinib, dexamethasone, and anti-infective therapy were given, and her condition remained stable after dexamethasone withdrawal. After 2 months of medication, she stopped using ruxolitinib. One week after stopping using ruxolitinib, she developed fever and died after 2 weeks of antibiotic therapy treatment in a local hospital. In conclusion, panniculitis and HPS are related in etiology, pathogenic mechanism and clinical manifestations. Abnormal activation of Janus-kinase and signal transduction activator of transcription pathway and abnormal release of inflammatory factors play an important role in the pathogenesis of the two diseases. The report suggests that ruxolitinib is effective and has broad prospects in the treatment of panniculitis associated HPS.