Mujer de 66 años con nódulos cutáneos recurrentes, esplenomegalia, anemia hemolítica y neumonía organizada / A 66-year-old woman with recurrent skin nodules, splenomegaly, hemolytic anemia, and organizing pneumonia

We present the case of a women previously diagnosed with nodularpanniculitis (biopsy compatible with neutrophilic dermatosis) andmultifactorial anemia with signs of hemolysis and splenomegaly,who refers reappearance of painful nodules in extremities and general syndrome. The differential diagnosis of the coexistence of these alterations is proposed, with the subsequent solution of the case. (AU)

Presentamos el caso de una mujer con diagnóstico previo de paniculitis nodular con biopsia compatible con dermatosis neutrofílicay anemia multifactorial con componente hemolítico asociada a esplenomegalia, que consulta por reaparición de nódulos dolorososen extremidades y síndrome general. Se plantea el diagnóstico diferencial de la aparición conjunta de estas alteraciones y posteriorresolución del caso clínico. (AU)

Weber-Christian disease: a case report.

Weber-Christian disease is a rare disease from the group of chronic fibrosing conditions characterized by inflamma-tion of the adipose tissue - panniculitis and fibrosing with frequent systemic manifestations. Etiopathogenesis of the disease is not fully known, participation of autoimmune mechanisms is anticipated. Here, we report a case of a patient with this rare disease, diagnosed after a long and demanding diagnostic process, including repeated lapa-rotomies. However, after immunosuppressive therapy, clinical and laboratory symptomatology improved rapidly as well as the patients quality of life. Key words: panniculitis - sclerosing mesenteritis - Weber-Christian disease.

Idiopatic lobular necrotizing paniculitis.

42-year old patient was presented to our clinic with a fever lasting for seven months and a ten month history of subcutaneous nodules on all extremities and trunk. Further examination revealed anaemia, lymphocytopenia and elevation of inflammatory parameters and liver enzymes. Authors comment their difficulties in differential diagnostic process. The bone marrow biopsy and reappraisal of subcutaneous lesions confirmed idiopathic lobular panniculitis, known as Weber-Christian disease. A combined immunosuppressive therapy was followed by improvement of clinical state as well as laboratory parameters (Tab. 2, Fig. 3, Ref. 11).

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Sterile nodular dermatitis in dogs.

The types of canine sterile nodular dermatitis discussed in this article have in common the clinical presentation of nodules or plaques; however, they differ in many aspects such as breed predilection, distribution and evolution of cutaneous lesions, systemic involvement, response to therapy, and prognosis. The definitive diagnosis should be based on multiple skin biopsy results. Other ancillary tests may be indicated in cases of systemic involvement. In addition, serum alpha1 antitrypsin can be measured to demonstrate the association between nodular panniculitis and serum alpha1 antitrypsin deficiency. A better understanding of the etiopathogenesis of each of these interesting skin conditions necessitates extensive and systematic diagnostic approaches.

[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.

Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis.

A 20-year-old Japanese man developed generalized, subcutaneous, painless nodules, fever, abnormal liver function, serosal effusions, hepatosplenomegaly, lymphadenopathy and anemia. Skin biopsies revealed lobular panniculitis with a morphologically benign histiocytic infiltration and prominent phagocytosis. Atypical T lymphocytes were also present in the skin and liver. The diagnosis given was aggressive cytophagic histiocytic panniculitis (CHP) or aggressive subcutaneous panniculitic T cell lymphoma (SPTCL). He received cyclophosphamide, doxorubicin, and vincristine on day 1, prednisolone on days 1-5, and etoposide on days 1, 3 and 5 (CHOP-E), with the support of granulocyte colony-stimulating factor. This regimen was repeated every 2 weeks and complete clinical remission (CCR) was attained after three cycles of CHOP-E. As the clinical course of aggressive CHP is recurrent and often fatal, he was given high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (APBSCT), after five cycles of CHOP-E. He has remained in CCR for 12 months after APBSCT. High-dose chemotherapy followed by APBSCT is considered to be one of the most beneficial therapies for patients with aggressive CHP and aggressive phase SPTCL.

[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.

Paniculitis nodular no supurativa o enfermedad de Weber Christian: a propósito del primer caso nacional en la infancia / Non suppurative nodular paniculitis or Weber Christian disease: apropos of the first national case in childhood

Se presenta el primer reporte en Uruguay de un caso de Paniculitis nodular no supurativa, o síndrome de Weber Christian. Esta es una enfermedad rara más aún en niños, y su etiología es desconocida. Esta se caracteriza clínicamente por nódulos subcutáneos, fiebre, repercusión general y manifestaciones sistémicas, sobre todo a nivel hepático, pulmonar y digestivo. Su diagnóstico es de sospecha clínica y confirmación anatomopatológica, con la biopsia de piel que evidencia una paniculitis lobulillar no supurativa característica. Se acompaña en ocasiones de alteraciones de la inmunidad celular. El tratamiento con corticoides es de relativo éxito, siendo el pronóstico incierto

Ten-year course of early-onset Weber-Christian syndrome with recurrent pneumonia: a suggestion for pathogenesis.

A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of fever with associated increase in the acute phase reactants, and the failure of the nonsteroidal anti-inflammatory drugs leads us to propose that this form of Weber-Christian syndrome reflects an inborn error in the regulation of the inflammatory response. Systematic investigation of this hypothesis could yield important information on the normal regulation of inflammation and could lead to a rational therapeutic approach to this puzzling and usually devastating illness.