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.

Enfermedad de Weber-Christian como causa infrecuente de hipertensión secundaria / Weber-Christian disease as unusual cause of secondary hypertension

Se describe el caso de un paciente varón de 53 años, diabético, afectado de paniculitis de Weber-Christian que cursa en forma de brotes recidivantes y con infiltración inflamatoria de la grasa perivascular, perirrenal y de los hilios renales; presenta HTA severa refractaria al tratamiento hipotensor múltiple. Pensamos que la severidad de las cifras de presión arterial pueda ser secundaria a la rigidez presente en el árbol vascular arterial provocado por la propia infiltración inflamatoria de la grasa a este nivel, pudiendo llegar a comportarse como una hipertensión renovascular donde el componente estenosante extrínseco sería dicha infiltración grasa periarterial. Se explica así la elevación de la tensión arterial sistólica, manteniendo la tensión arterial diastólica dentro de los parámetros de la normalidad. A pesar de la baja prevalencia, la paniculitis debería ser tenida en cuenta como causa de hipertensión refractaria en general e incluso de hipertensión renovascular en particular (AU)

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.

Angiopaniculitis granulomatosa mamaria / Mammary granulomatous angiopanniculitis

Introducción Las lesiones granulomatosas en la mama constituyen un variado espectro de alteracione que pueden traducir diferentes tipos de afecciones. Material y método: Presentamos una observación clinicopatológica acerca de una paciente de 49 años de edad que mostró lesiones recurrentes, primero en las mamas y más tarde en el muslo y pierna, que se resolvieron de forma espontánea sin ningún tratamiento. Resultados: EL examen citológico por punción de todas las lesiones mostró granulomas no necronizantes. El estudio histológico de la lesión mamaria evidenció una angiopaniculitis granulomatosa mamaria, consistente en la asociación de una angeítis llinfoide y una paniculitis granulomatosa no necronizante. Conclusión: La angiopaniculitis granulomatosa mamaria debe diferenciarse de las llamadas mastitis granulomatosas idiopáticas. El proceso puede simular clínica y radiográficamente un proceso neoplásico. Su curso evolutivo, a pesar de su carácter recurrente, es benigno, siendo considerada por algunos autores como una variedad de la enfermedad de Weber-Christian (AU)