Pancreatic panniculitis (PP) is a necrotizing inflammation of subcutaneous fat that is a rare complication of pancreatic disease appearing in 2% to 3% of all patients. It is more common in the elderly and often affects the extremities. It presents as skin inflammation with pain and erythema nodules. We report a case of acute pancreatitis associated with PP in an old female. She was admitted for vomiting and abdominal pain for 3 days and presented with a 2-week history of erythematous subcutaneous nodules on her legs. Laboratory and ultrasonic findings revealed acute pancreatitis. High frequency ultrasound showed hypoechoic foci in subcutaneous soft tissue layer and adipose layer. Histopathological examination confirmed the diagnosis of PP. Nodules disappeared with the resolution of acute pancreatic inflammation. PP may be the first manifestation of pancreatic disease. Imaging features of this pathology are seldom described and ultrasonic diagnosis experience is worth to be accumulated.
Acute Disease ; Aged ; Female ; Humans ; Inflammation/complications* ; Pancreatic Diseases/diagnostic imaging* ; Pancreatitis/diagnostic imaging* ; Panniculitis/etiology* ; Ultrasonography

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.
Humans ; Male ; Female ; Middle Aged ; Aged ; Lymphohistiocytosis, Hemophagocytic/drug therapy* ; Glucocorticoids/therapeutic use* ; Epstein-Barr Virus Infections/complications* ; Etoposide/therapeutic use* ; Prednisone/therapeutic use* ; Herpesvirus 4, Human ; Panniculitis/complications* ; Dexamethasone/therapeutic use* ; Exanthema/complications* ; Ferritins/therapeutic use* ; Anti-Bacterial Agents/therapeutic use* ; Fibrinogen/therapeutic use*

Acute Disease ; Aged ; Amylases ; blood ; Humans ; Lipase ; blood ; Magnetic Resonance Imaging ; Male ; Pancreatitis ; complications ; diagnosis ; diagnostic imaging ; Pancreatitis, Chronic ; complications ; diagnosis ; Panniculitis ; diagnosis ; diagnostic imaging ; etiology ; pathology

Sclerosing mesenteritis (SM) is a rare disease characterized by chronic nonspecific mesenteric inflammation and fibrosis of unknown etiology. Some tumefactive SM shows diffuse accumulation of IgG4-positive plasma cells and is considered as a part of the spectrum of IgG4-related disease. An association between inflammatory bowel disease and IgG4-related disease has been indicated. A 45-year-old woman visited our hospital due to weight loss with intermittent lower abdominal discomfort. Pelvic ultrasound revealed a mass-like lesion in the abdominal wall and pelvis MRI demonstrated a 5.9 cm sized wall-enhancing mass with heterogeneous signal intensity from right adnexa to the abdominal wall. Tumor resection and adhesiolysis was done because of severe adhesion with the small bowel, colon, bladder, uterus, and abdominal wall. Appendectomy was also performed due to adhesion and edematous change. Histological examination of the resected mass showed findings that were compatible with IgG4-related SM. The resected appendix showed chronic granulomatous inflammation without evidence of tuberculosis. She was diagnosed with Crohn's disease after undergoing colonoscopy and CT enterography. Herein, we report a rare case of IgG4-related SM that occurred in conjunction with Crohn's disease.
Anti-Inflammatory Agents/therapeutic use ; Appendix/pathology ; Azathioprine/therapeutic use ; Colonoscopy ; Crohn Disease/complications/*diagnosis/drug therapy ; Female ; Humans ; Immunoglobulin G/*blood ; Magnetic Resonance Imaging ; Mesalamine/therapeutic use ; Middle Aged ; Panniculitis, Peritoneal/*diagnosis/etiology/ultrasonography ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed ; Urinary Bladder/pathology

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.
Anti-Inflammatory Agents/therapeutic use ; Antineoplastic Agents, Hormonal/therapeutic use ; Diagnosis, Differential ; Humans ; Laparoscopy ; Male ; Middle Aged ; Panniculitis, Peritoneal/complications/*diagnosis/drug therapy ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/complications/*diagnosis/pathology ; Tamoxifen/therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo explore the clinical presentation, treatment and prognosis study of primary subcutaneous panniculitis-like T-cell cutaneous lymphoma (SCPTCL).

METHODSTen cases of SCPTCL, treated in our hospital from January 1999 to January 2009, were included in this study. Their clinicopathological data were reviewed and analyzed retrospectively.

RESULTSthe median age was 50.5 years (range: 10 - 58), 4 males and 6 females. There were seven CD56 positive, two negative cases and 1 unclear case. Four cases had repeatedly nodules regressed spontaneously without treatment before diagnosis and new nodules appeared at different sites. Seven patients presented with multiple subcutaneous nodules or deeply seated plaques, most commonly on the extremities and trunk. Ulceration of nodules occurred in 3 cases, and the lesions were painful in five cases. The lesions appeared nodules at the beginning, and then gradually grew into tumors. Four patients had abnormal liver function and one patient had hemophagocytic syndrome (HPS), four patients had lymphadenopathy or visceral involvement. Three cases with single lesion underwent surgical excision in combination with chemotherapy or chemotherapy/radiotherapy. One case lost follow up, and two cases live without disease. Among the seven patients with multiple lesions, lymphadenopathy or visceral involvement, one underwent local surgical excision and is alive without disease, six of them received chemotherapy or multi-modality treatment mainly with chemotherapy. Three of these 6 cases are alive without progression, one used histone deacetylase inhibitors after progression and obtained partial regression, and 2 died. The median follow-up for all the 10 patients was 44 months (range: 14 - 99). The progression free survival was 66.7% (6/9), and overall survival was 77.8% (7/9).

CONCLUSIONSPTCL has an indolent course, some lesions can regress spontaneously and relapse again. Patients with single lesion may live long-term without disease after multimodality therapy. Patients with multiple lesions or extracutaneous involvement are sensitive to CHOP-like regimen, but the duration of remission is short. Histone deacetylase inhibitors may be a promising drug in the treatment for SCPTCL relapse.

Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD56 Antigen ; metabolism ; Child ; Combined Modality Therapy ; Cyclophosphamide ; therapeutic use ; Disease-Free Survival ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Lymphatic Diseases ; etiology ; Lymphohistiocytosis, Hemophagocytic ; etiology ; Lymphoma, T-Cell ; complications ; drug therapy ; metabolism ; radiotherapy ; surgery ; Male ; Middle Aged ; Panniculitis ; complications ; drug therapy ; metabolism ; radiotherapy ; surgery ; Prednisone ; therapeutic use ; Retrospective Studies ; Survival Rate ; Vincristine ; therapeutic use ; Young Adult

Anti-Inflammatory Agents ; therapeutic use ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Ear Cartilage ; Female ; Humans ; Integrative Medicine ; methods ; Middle Aged ; Panniculitis ; complications ; drug therapy ; Phytotherapy ; Polychondritis, Relapsing ; complications ; drug therapy ; Prednisone ; therapeutic use ; Treatment Outcome

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic diseases; these diseases include acute and chronic pancreatitis, pancreatic carcinoma, pseudocyst, and other pancreatic diseases. This malady is manifested as tender erythematous nodules on the legs, buttock, or trunk. Histopathologically, it shows the pathognomonic findings of focal subcutaneous fat necrosis and ghost-like anucleated cells with a thick shadowy wall. We herein report a case of fatal pancreatic panniculitis that was associated with acute pancreatitis in a 50-yr-old man. He presented with a 3-week history of multiple tender skin nodules, abdominal pain and distension. Laboratory and radiologic findings revealed acute pancreatitis, and skin biopsy showed pancreatic panniculitis. Despite intensive medical care, he died of multi-organ failure 3 weeks after presentation.
Acute Disease ; Biopsy ; Erythema/pathology ; Fatal Outcome ; Humans ; Male ; Middle Aged ; Necrosis ; Pancreas/pathology ; Pancreatitis/*complications/*mortality ; Pancreatitis, Alcoholic/diagnosis/pathology ; Panniculitis/*complications/*mortality ; Skin/pathology ; Skin Diseases/diagnosis/pathology ; Tomography, X-Ray Computed/methods

BCG Vaccine ; adverse effects ; Humans ; Immunologic Deficiency Syndromes ; complications ; Panniculitis ; etiology ; T-Lymphocytes ; Tuberculosis ; etiology

Child ; Humans ; Lymphoma, T-Cell ; complications ; Panniculitis ; etiology ; Subcutaneous Tissue