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.

Genetic study of a family with two members with Weber Christian disease (panniculitis) and alpha 1 antitrypsin deficiency.

Alpha 1 antitrypsin phenotypes and serum levels are presented for a family in which two brothers have Weber Christian disease and alpha 1 antitrypsin (PI) Z phenotypes. Clinical histories are described for these two men. A younger brother has the PI Z phenotype but does not have the disease, indicating that additional genetic and/or environmental factors contribute to the pathogenesis of Weber Christian disease. However, the two cases described provide additional evidence for a relationship between alpha 1 antitrypsin deficiency and the development of symptoms.

A case of cytophagic histiocytic panniculitis: successful treatment of recurrent attacks with steroid pulse therapy and oral cyclosporin A.

We report a 35-year-old man, who had been diagnosed with Weber-Christian disease, presented with acute onset of high fever, malaise, jaundice and hepatosplenomegaly with subcutaneous nodules. Laboratory tests showed elevated serum ferritin and liver enzymes, especially lactate dehydrogenase (LDH), with pancytopenia and coagulation abnormalities. Peripheral blood and bone marrow examinations showed erythro-, leuko- and thrombo-phagocytic histiocytes and macrophages. The patient developed the same clinical features seven years ago. Based on diagnosis of cytophagic histiocytic panniculitis, the patient was treated with steroid pulse therapy and oral cyclosporin A. The combination therapy caused a marked improvement in the clinical condition.

[Weber-Christian disease with systemic involvement. Apropos of a case of histiocytic cytophagous panniculitis]. / La malattia di Weber-Christian ad interessamento sistemico. A proposito di un caso di pannicolite istiocitica-citofagica.

A case of systemic Weber-Christian disease involving the liver and medulla, with pancytopenia and disseminated intravascular coagulation (DIC) is reported. Clinical and histopathological diagnostic criteria are stressed. Post-mortem findings are described.

[Successful treatment of Weber-Christian panniculitis with cyclosporin-A]. / Weber-Christian panniculitis eredményes kezelése ciclosporin A-val.

The inflammatory disease of the subcutaneous fatty tissue are called panniculitis. The panniculitis has got a septal and a lobular histopathologic type. In the opinion of the etiologic classification occurs idiopathic (primary) or secondary entity. The secondary illnesses develop as the result of physical, chemical, biochemical, immunological, degenerative and proliferative reactions. In a part of panniculitis the etiologic factor is unknown. The authors demonstrate a rare type of the panniculitis, the Weber-Christian syndrome. This disease is an acute relapsing panniculitis with unknown etiology, fever, general symptoms and typical subcutaneous nodes. In their case the histological examination of the subcutaneous node resulted in non-suppurative, lobular panniculitis. The oral metilprednisolon and the occurring twice parenteral cyclophosphamide treatment had been proved to be unsuccessful, for this reason they started cyclosporin A therapy. Because of the cyclosporin A treatment the subcutaneous nodes regressed, the general symptoms ceased and the pathological laboratory findings became normal. The patient is asymptomatic under permanent therapy now. The authors discuss the differential diagnostic of this illness and the ways of treatments.

Alpha 1-antitrypsin deficiency associated with panniculitis.

Alpha 1-Antitrypsin is the principal serum protease inhibitor. In addition to the well-recognized association with early-onset emphysema and cirrhosis, alpha 1-antitrypsin deficiency may be associated with panniculitis. In this article we describe three patients in whom the recognition of certain clinical and histologic features of panniculitis eventually led to the diagnosis of alpha 1-antitrypsin deficiency. Two of our patients were young adults and one was a child. All three had draining, panniculitis, or cellulitis-like lesions at sites of prior trauma. The histopathologic findings were characterized by liquefactive dermal necrosis and collagenolysis of the fibrous septa of the subcutis. The combination of these clinical and microscopic findings should suggest the diagnosis of alpha 1-antitrypsin deficiency panniculitis. The suspicion can be verified by obtaining quantitative serum levels and enzyme phenotyping. The identification of the alpha 1-antitrypsin deficiency state as the cause of a distinct type of panniculitis adds additional evidence for the elimination of the term Weber-Christian disease.

Systemic Weber-Christian disease: a case report with lipoprotein profile and immunological evaluation.

A case of fatal systemic Weber-Christian disease is described. In vitro immunological function and lipoprotein measurements are presented with a subsequent discussion on the possible aetiology of the condition.

Arthropathy, skin and bone lesions in pancreatic disease.

A patient with a history of alcoholism and pancreatic calcification, developed subcutaneous fat necrosis and an arthropathy, associated with a pancreatic pseudocyst and accompanied by an elevation of serum lipase and amylase. The illness was complicated by bacteremia and destructive bone lesions. A clinical distinction between osteomyelitis and medullary fat necrosis proved difficult. Infection of bone was demonstrated at one site but did not exclude medullary fat necrosis elsewhere.

Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease.

Hyperlipidemia of initially Type V and finally of Type III was observed in a patient with Weber-Christian disease. The lipoprotein lipase (LpL) and hepatic triglyceride lipase (HTGL) activities of the post-heparin plasma were low, but detectable. Both lipase activities were higher when assayed with 20 microliters of post-heparin plasma than with more than 30 microliters, indicating the presence of some inhibitory factor in the plasma. This plasma inhibited purified LpL and HTGL from human post-heparin plasma. Zonal ultracentrifugation studies showed that the inhibitor of hepatic lipase was associated with the middle fraction of intermediate lipoprotein and low density lipoproteins (1.020 less than d less than 1.040). These results are consistent with the idea that dyslipoproteinemia in this patient was partially due to dysfunction of the catabolic system caused with an inhibitor of lipolytic enzymes.

Possible mechanisms of glucocorticoid--unresponsive pyrexia. Defect in lipocortin 1?

Glucocorticoids have a strong anti-inflammatory action, and are indispensable in the treatment of inflammatory diseases. We had a patient with the Weber-Christian disease having an intractable high fever that did not respond to even a high-dose glucocorticoid therapy, but was responsive to a nonsteroidal antiinflammatory drug. To elucidate possible mechanisms of the glucocorticoid-unresponsive fever, we have investigated the in vitro production of two eicosanoids, prostaglandin (PG)E2 and leukotriene (LT)B4, from the peripheral blood polymorphonuclear leukocytes after stimulation by ionophore A23187. The patient's leukocytes produced much larger amount of PGE2, but the same amount of LTB4, as did those of two control groups. More interestingly, the production of eicosanoids was inhibited by dexamethasone less in the patients than in the controls. Indomethacin suppressed the production of PGE2 both in the patients and in the controls. These results might be relevant in the glucocorticoid-unresponsive pyrexia.