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

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

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Adult ; Biopsy ; Case Report ; Exophthalmos/pathology* ; Exophthalmos/etiology* ; Exophthalmos/drug therapy ; Glucocorticoids/administration & dosage ; Human ; Magnetic Resonance Imaging ; Male ; Panniculitis, Nodular Nonsuppurative/pathology* ; Panniculitis, Nodular Nonsuppurative/drug therapy ; Panniculitis, Nodular Nonsuppurative/complications*

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.
Adult ; Biopsy ; Case Report ; Exophthalmos/pathology* ; Exophthalmos/etiology* ; Exophthalmos/drug therapy ; Glucocorticoids/administration & dosage ; Human ; Magnetic Resonance Imaging ; Male ; Panniculitis, Nodular Nonsuppurative/pathology* ; Panniculitis, Nodular Nonsuppurative/drug therapy ; Panniculitis, Nodular Nonsuppurative/complications*

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

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

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