An unreported case of pancreatic panniculitis in a liver transplant patient.

Pancreatic panniculitis is an uncommon condition that can occur in association with pancreatic disease. Most of the cases reported to date were associated with acute or chronic pancreatitis and pancreas cancer. Recently, development has been described in kidney transplant patients and secondarily to allograft pancreatitis in a pancreas-kidney transplant recipient. Both findings suggest that immunological processes may be involved in the pathogenesis of this entity. We report for the first time a case of acute pancreatitis associated with pancreatic panniculitis in a patient who underwent a liver transplant 10 months before. A 69-year-old man with a history of epigastric pain of a few days of evolution was presented with painful subcutaneous nodules on both legs. Blood chemistry showed raised serum amylase and lipase levels. Ultrasonography and multislice CT scan were suggestive of an acute pancreatitis. A skin biopsy showed typical features of pancreatic panniculitis which included lobular panniculitis with lipocyte degeneration with ghost cells. The administration of octreotide resulted in both a rapid improvement of symptoms and a disappearance of skin lesions. Liver transplant specialists should be aware that the pancreatic panniculitis could be a manifestation ofpancreas disease in patients who have undergone l ver transplantation.

A case of Weber-Christian disease with later development of rheumatoid arthritis.

Weber-Christian disease (WCD) is a syndrome characterized by recurrent subcutaneous nodules, fever, occasional lipoatrophy, fatigue, arthralgia, and myalgia. We report a case of WCD associated with rheumatoid arthritis. A 65-year-old woman consulted our outpatient clinic because of bilateral hand swelling. The patient had presented with fever and subcutaneous nodules in her trunk and upper and lower extremities in 1983. At that time, the dermatology department diagnosed this patient as having WCD after biopsy of the nodules demonstrated lobular panniculitis. She has been treated with corticosteroid (5-15 mg/day) since then. The patient continued to have recurrent episodes of transient inflammatory arthritis in the small joints of the fingers and fever, and was initially assessed at our institution in October 2007. Finally, in November 2007, she was diagnosed as having both WCD and rheumatoid arthritis (RA) and treated with corticosteroid (5 mg/day) and methotrexate (MTX) (7.5 mg/week). Thereafter, her clinical symptoms gradually improved. This is the second case of WCD showing the subsequent development of RA, successfully treated with MTX, in the English literature. This case may provide clinical insight into WCD and RA.

[Weber-Christian disease: unknown etiology systemic panniculitis]. / Doença de Weber-Christian: paniculite sistémica de etiologia desconhecida.

Panniculitis is an inflammation occurring within adipose tissue. This process is often associated with auto-immune diseases, infections, malignancy and others diseases of unknown cause. We report a 16-year-old woman with constitutional symptoms and relapsing nonsuppurative nodular panniculitis. The etiological study didn't identify any associated disease, on admission or in 2,5 years of follow-up. This is a case of Weber-Christian disease with a favourable corticosteroid therapy result. The authors present a Weber-Christian disease bibliographic revision.

Pancreatic panniculitis associated with allograft pancreatitis and rejection in a simultaneous pancreas-kidney transplant recipient.

Pancreatic panniculitis is an uncommon condition that can occur in association with pancreatic disease. We present a case of pancreatic panniculitis in a female pancreas-kidney transplant recipient 5 months post-transplant. The patient was on standard immunosuppressive medications and had acute rejection of her renal allograft. The diagnosis of allograft pancreatitis and rejection presenting with pancreatic panniculitis was supported clinically, histopathologically and by laboratory and imaging data. This is the fourth case of pancreatic panniculitis occurring in a transplant recipient and the first in a simultaneous pancreas-kidney transplant recipient. It is also the first case associated with allograft rejection. Clinicians should be aware that pancreatic panniculitis may be a manifestation of underlying allograft pancreatic disease.

[Nodular panniculitis--a paraneoplastic syndrome in pancreatic carcinoma]. / Noduläre Pannikulitis--ein paraneoplastisches Syndrom bei Pankreaskarzinom.

A 68-year-old woman is described, who suffered from painful, subcutaneous nodules on her lower extremities. The clinical investigations revealed a solitary liver metastasis of a pancreatic carcinoma removed seven years before. Her serum lipase was 6088 U/l (normal value < 190 U/l). After embolisation and surgical resection of the metastasis the serum lipase level became unmeasurably low and the painful nodular panniculitis disappeared completely. Nodular panniculitis is a paraneoplastic syndrome not well known among physicians, which was easily treatable in our case.

Pfeifer-Weber-Christian's panniculitis in an obese patient with antinuclear antibody-positive leukocytoclastic vasculitis.

Pfeifer-Weber-Christian's panniculitis is a rare syndrome characterized by fever, arthralgias, fatigue and recurrent nodular panniculitis. It has been associated with pancreatic diseases, trauma, connective tissue diseases, alpha-1-antitrypsin deficiency, systemic lupus erythematosus, infections, lymphoproliferative diseases and neoplasias. We report the case of a 43-year-old obese male patient who presented with asthenia, arthralgias, intermittent fever, skin erythema and a large hard-elastic tumor of the right calf. Laboratory analysis revealed increased values of the immunophlogosis parameters and positivity for serum antinuclear antibodies. Surgical drainage of the abscess-like tumor mass, revealed leakage of a sterile, subflavious, oily and thick liquid; a skin biopsy showed intra and perivascular infiltration by neutrophils, diagnostic for leukocytoclastic vasculitis. Treatment with prednisone induced clinical improvement and normalization of the laboratory data. The clinical picture, laboratory data and efficacy of prednisone therapy confirmed that the patient developed Pfeifer-Weber-Christian's panniculitis in the clinical setting of an antinuclear antibody-positive leukocytoclastic vasculitis.

Clinicopathologic evaluation of nodular cutaneous lesions of Behçet syndrome.

Among the cutaneous manifestations, nodular lesions are rather common in Behçet syndrome. The histologic nature of these lesions has been a matter of controversy. To establish their distinguishing features, biopsy specimens from nodular lesions of 24 patients with Behçet syndrome, 25 with nodular vasculitis (NV), and 20 with erythema nodosum (EN) were compared. Statistical analysis revealed insignificant differences between most of the histologic features of Behçed syndrome and NV. However, neutrophil-predominating infiltrate in the subcutis was more common in Behçet syndrome, while necrosis and granuloma formation were encountered more frequently in NV. The differences between Behçed syndrome and EN were more significant. Septal panniculitis, lymphocyte-predominating infiltrate, absence of many vascular changes as well as vasculitis, and necrosis were features in favor of EN. Nodular lesions of Behçet syndrome are mainly neutrophilic vascular reactions with histologic features similar to NV but significantly differing from EN associated with other systemic diseases.

A case of cytophagic histiocytic panniculitis associated with exertional rhabdomyolysis.

An 18-year-old man who suffered from panniculitis involving the entire left lower limb after exertional rhabdomyolysis is reported. A high fever (>39 degrees C) and leukocytosis (>20,000/microL) persisted for 1 week, and his general status deteriorated rapidly into pre-disseminated intravascular coagulation, complicated by pleural effusion and prolonged clotting time. His condition was dramatically improved by steroid pulse therapy and he has remained in good health for the 20 months since discharge. Histologic examination of subcutaneous tissue from the swollen left lower limb revealed pleomorphic small, medium or large lymphocytes, macrophages and neutrophils infiltrating the edematous subcutaneous adipose tissue in a lobular panniculitis-like pattern. The majority of inflammatory cells were T lymphocytes, with equal proportions of CD4+ and CD8+ cells. As polymerase chain reaction did not show bands suggesting T cell receptor gamma gene rearrangement, the proliferation of T lymphocytes was considered to be polyclonal. The T lymphocytes also expressed Fas ligand, suggesting the involvement of Fas-mediated cytotoxicity. This case may represent a new category of cytophagic histiocytic panniculitis induced by exertional rhabdomyolysis.

Enfermedad de Weber-Christian: a propósito de un caso / Weber-Christian disease: a proposal of case

La paniculitis lobular idiopática, también denominada paniculitis nodular febril recurrente, o enfermedad de Weber-Christian, es el término empleado para describir un grupo de enfermedades que se manifiestan clínicamente por nódulos subcutáneos inflamatorios. Enfermedad de Weber-Christian es el término aplicado a los casos idiopáticos de paniculitis lobular asociada con síntomas sistémicos. Se presenta el caso de un paciente con síndrome febril muy intenso acompañado de lesiones en piel, las que aparecen sin causa alguna, y luego desaparecen sin tratamiento, asociado a varios síntomas sistémicos. Se detalla el caso y se hace una revisión de todo lo publicado en la literatura médica a propósito de esta enfermedad

[Hepatic artery ligation for liver metastasis of a acinar cell carcinoma of the pancreas revealed by a nodular panniculitis]. / Ligature de l'artère hépatique pour métastases hépatiques d'un carcinome à cellules acineuses du pancréas révélé par une panniculite nodulaire.

We report the case of a 67-year-old-man presenting with pancreatic acinar cell carcinoma revealed by dermatological manifestations of cytosteatonecrosis and treated by hepatic artery ligation. The pancreatic etiology of these lesions was suspected due to hyperlipasemia, and was confirmed by abdominal computerized tomography showing a pancreatic tumor and multiple liver nodules, and by histological examination of one of these lesions. Because of symptomatic treatment failure, rapid impairment of patient's general condition, and by analogy with the treatment of hepatic metastases of neuroendocrine tumors, hepatic artery ligation was performed. Lipasemia decreased markedly and symptoms disappeared for 45 days. Hepatic artery obstruction may be used for emergency treatment of secreting liver metastases.

[Endoscopic treatment of subcutaneous fat necrosis secondary to pancreatic-vascular fistula: apropos of a case]. / Traitement endoscopique d'une cytostéatonécrose secondaire à une fistule pancréaticovasculaire: à propos d'un cas.

PATIENT AND METHOD: We report the case of a 68-year-old man who presented a pancreatic tumor with a pancreato-vascular fistula and a Weber-Christian syndrome. Pancreatic enzymes levels at the admission were high: amylasemia 2,470 IU/L (N < 110) and lipasemia 11,700 IU/L (N < 220). The treatment consisted in total parenteral nutrition and somatostatin (100 micrograms x 3/day). Because we noted neither clinical nor biological improvement after 10 days of treatment, we performed an endoscopic retrograde pancreatography. During this examination, we put a 7 French diameter prosthesis through the Wirsung stenosis. RESULTS: No problem arose after endoscopy: since the day after the endoscopy, pancreatic enzymes decreased by half and become normal in 4 days; arthralgias and cutaneous injuries, both caused by cytosteatonecrosis, disappeared respectively in 5 and 10 days. There is no evidence of subsequent recurrence after 3 months of follow-up. CONCLUSION: Pancreatic endoscopic prosthesis can replace the surgical treatment of pancreato-vascular fistula with a good efficacy.

Panniculitis: recent developments and observations.

Dermatopathologists rarely greet a biopsy of panniculitis with total confidence that a specific, definitive diagnosis will be rendered. As with many other areas in dermatopathology, our understanding of the pathogenesis of many forms of panniculitis is incomplete. This article examines a subset of panniculitis primarily from a pathogenetic standpoint, with the intention of providing a differential diagnosis for those cases in which ischemic changes are seen in the subcutis. The diverse group of conditions evoked by this approach also shares the distinction of having been the focus of nosologic and causative controversy, both historically and currently. In particular, stasis-associated sclerosing panniculitis, vascular calcification-cutaneous necrosis syndrome (calciphylaxis), oxalosis, and nodular vasculitis-erythema induratum are examined in depth. Erythema nodosum and variants, other granulomatous panniculitides, and panniculitides showing cytophagocytosis are also discussed with current perspectives.

[Scrotal panniculitis due to cold: a pseudo-tumoral lesion in the prepubertal child. Report of a case]. / Panniculite scrotale au froid: une lésion pseudo-tumorale chez l'enfant prépubère. A propos d'une observation.

We reported a case of scrotal panniculitis in a 7 year-old boy after exposure to cold by swimming in cold sea water. Scrotal cold panniculitis is an unusual, confined to prepuberal patients. This entity must be known to avoid surgical exploration because injuries subside spontaneously within few weeks.

Nodular intra-abdominal panniculitis: an accompaniment of colorectal carcinoma and diverticular disease.

Intra-abdominal panniculitis is a tumour-like inflammatory condition of adipose tissue. The aetiology and pathogenesis of the disease is unknown, but a number of associated diseases have been recorded. It has been customary to deal with only primary cases in the literature. This study was undertaken in order to describe the entire spectrum of the disease including primary as well as secondary cases. Eleven patients are reported, nine of which had an associated colorectal disease in direct continuity with areas of intra-abdominal panniculitis. It is concluded that intra-abdominal panniculitis should not be regarded as a specific nosological entity but merely a result of injury to the fat cells. Intra-abdominal panniculitis is seen more often as a secondary local phenomenon than as a primary condition, and in both cases it is associated with considerable differential diagnostic problems. Primary diseases involved are, among others, colorectal carcinoma and diverticulosis. The aetiologic agent(s) are still unknown, but substances liberated from a damaged bowel might play a pathogenetic role.

[Pancreatogenic panniculitis]. / Pancreatogene panniculitis.

A 40-year-old woman who had been treated for mesangiocapillary glomerulonephritis by renal transplantation, and who still used furosemide, azathioprine, cyclosporine and prednisone, was admitted with multiple erythematous nodes on the lower legs. Pancreatogenic panniculitis caused by pancreatitis was diagnosed. The patient died with a haemorrhagic and necrotic pancreas one month after admission. Pancreatogenic panniculitis occurs in 2-3% of patients with pancreatitis or pancreatic carcinoma. It can be a first manifestation of an undetected pancreatic disease.