Weber-Christian panniculitis and auto-immune disease: a case report.

A case is described of Weber-Christian panniculitis accompanied by a gammaglobulin disturbance which preceded by five years the diagnosis of an autoimmune hepatitis and pancytopenia. Also associated was the onset of diabetes mellitus, found at necropsy to be related to pancreatic islet amyloid deposition. This case reinforces the view that Weber-Christian panniculitis may be an adipose response to a variety of immunological stimuli.     

Systemic Weber-Christian disease

A patient suffering from Weber-Christian panniculitis was found at necropsy to have similar lesions in the visceral adipose tissue. When the lesions occur internally, diagnosis during life is complicated by the difficulty of examining visceral adipose tissue and the disease may often go unrecognized, but patients suffering from Weber-Christian panniculitis should be investigated bearing in mind the possibility of internal lesions. Only 11 cases confirmed by necropsy appear to have been reported hitherto. The aetiology remains obscure. It is suggested that the name `systemic Weber-Christian disease' be applied when the lesions occur in adipose tissue other than the panniculus adiposus and that the term `Weber-Christian panniculitis' be used when the lesions are confined to the subcutaneous adipose tissue.     

Weber-christian panniculitis with systemic cytophagic histiocytosis

Summary A 49-year-old woman suffered from Weber-Christian panniculitis with a typically periodic course. Subsequently, the attacks of the disease developed to a severe state: high fever, endotoxinemia, pancytopenia, and clotting disorder, in addition to the cutaneous manifestations. One and a half years after the outbreak of the disease the patient died in a septic shock. At autopsy all three stages of Weber-Christian panniculitis were found. In addition, an immense proliferation of benign cytophagic histiocytes could be observed in the bone marrow, spleen, lymph nodes and, less distinctly, in the fatty and interstitial tissues of the visceral organs. As a nosologic entity, the Weber-Christian disease is frequently questioned. Some authors consider the lobular, histiocytic, cytophagic panniculitis a unique syndrome. On the other hand, it must be emphasized that benign, cytophagic histiocytosis may exist associated with infections or may accompany different diseases. Furthermore, the Weber-Christian panniculitis is clinically and morphologically well defined.Abbreviations PAP method peroxydase-anti-peroxydase method     

AN UNUSUAL CASE WHICH BEGAN WITH SUBCUTANEOUS PANNICULITIS FOLLOWED BY FEVER, SEVERE HEPATIC INVOLVEMENT AND HYPERLIPIDEMIA

A 25-year-old man was at first dermatologically suspected as suffering from Weber-Christian syndrome because of subcutaneous panniculitis, but his skin lesions disappeared completely during the course. Hyperlipidemla, disturbances In liver function, and leukemoid reaction became remarkable, and he died of subarachnoid hemorrhage eleven months after onset. Necropsy revealed subarachnoid hemorrhage at the base of the brain, lipogranulomatous and inflammatory lesions in the upper lobe of the left lung, a remarkable fatty liver, splenomegaly, pericarditis, and foam cells in the spleen, liver, and bone marrow. A comparison with 57 autopsy cases of Weber-Christian syndrome reported in the literature showed our case to be an exceptional instance of Weber-Christian syndrome, If the present case is not to be regarded as a different disease entity. ACTA PATH. JAP. 27:213–224, 1977.     

Weber-Christian disease presenting with proptosis: a case report

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.     

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.     

POST-STEROID PANNICULITIS WITH CEREBRAL VASCULAR and PULMONARY COMPLICATIONS—REPORT OF AN AUTOPSY CASE

An autopsy case report was made on a 28-year-old female who had systemic nodular panniculitis, necrotizing arteritis of the cerebral artery and unusual pulmonary lipogranuloma after massive, long-term administration of corticosteroids for nephrotic syndrome. Panniculitis in this case was fairly consistent with the category of post-steroid panniculitis and was thought to be the first adult case, of post-steroid panniculitis developing in the internal as well as subcutaneous adipose tissue. The other two lesions in the brain and the lung above mentioned might be also regarded as adverse effects of corticosteroid therapy. The subcutaneous nodular lesion was studied chemically and histochemically, and the genesis of nodular panniculitis in this case was discussed as compared with sclerema neonatorum.     

Systemic nodular panniculitis with cardiac involvement

A case of systemic nodular panniculitis is described in which the myocardium was found at necropsy to be extensively involved with focal interstitial carditis, identical histologically with nodules of panniculitis biopsied from the skin. This degree of myocardial involvement, which was not apparent during life and was not confined to pericardial or myocardial adipose tissue, has not previously been reported. The literature relating to nodular panniculitis is briefly reviewed and the concept of Weber-Christian disease critically re-appraised.     

A case of mesenteric panniculitis in a patient with Tangier disease

Mesenteric panniculitis is a rare disease caused by idiopathic inflammation of adipose tissue, most commonly affecting the mesentery of the small bowel. We present a unique case of mesenteric panniculitis in a patient with Tangier disease; a rare genetic disorder caused by mutations in the ABCA1 gene, leading to deficiency of high-density lipoprotein in the blood and accumulation of cholesterol esters within various tissues. The accumulation of cholesterol esters in body tissues in patients with Tangier disease may contribute to the pathogenesis of mesenteric panniculitis; although there is limited evidence to support this hypothesis due to the rarity of concurrent disease.     

Severe neonatal-onset panniculitis in a female infant with Prader-Willi syndrome

The panniculitides are a group of heterogeneous inflammatory diseases involving the subcutaneous fat, the pathogenesis of which is poorly understood. Here, we report on a female infant with Prader-Willi syndrome who developed a systemic inflammatory disorder in the neonatal period demonstrating recurrent panniculitis as a prominent feature. This is the second report of an association between Prader-Willi syndrome and panniculitis. Such an association might be explained by the unmasking of a recessive allele as a consequence of hemizygosity, in the case of a 15q11 deletion, or homozygosity, in the case of maternal isodisomy.     

Case of subcutaneous and mesenteric acute panniculitis with Sj?gren's syndrome]

We report a case of a 27-year-old Japanese female with Sjogren's syndrome (SS), who suffered from several episodes of subcutaneous and mesenteric panniculitis with a recurrence within one year. After a history of fever and skin rash, the patient underwent surgery at a local hospital with a diagnosis of acute appendicitis complicated with an ileocecal abscess. She was also diagnosed as having SS. After the operation, the fever and skin rash persisted. She was treated with prednisolone (PSL), and her symptoms resolved. A recurrent bout of abdominal pain with fever, annular erythema on the trunk and a nodular erythematous rash on the lower extremities occurred six months after the operation. A skin biopsy from the lower extremities showed findings that were compatible with panniculitis. Abdominal computer tomography (CT) showed a diffuse swelling with soft tissue density in the intestinal mesenterium and para aortic area. A retrospective examination of the operative specimen obtained from the local hospital revealed centrilobular infiltration of neutrophils in the mesenteric adipose tissue with fat necrosis, which is compatible with mesenteric panniculitis. Twenty mg/day of PSL was successful in treating the systemic panniculitis, and the abnormal diffuse soft tissue density on the abdominal CT disappeared after three weeks of PSL administration. Systemic panniculitis is a rare complication in SS, and the pathogenesis is unclear.     

A 38-year old woman with a dural based lesion

Isolated intracranial xanthogranulomas arising from the dura mater are extremely rare.We present a case of a symptomatic large right frontoparietal dura based intracranial xanthogranuloma in a 38-year-old female. Xanthogranulomas are benign non-Langerhans cell histiocytic lesions. They are frequently described in the skin of infants and children. Extracutaneous manifestations especially in the central nervous system are highly uncommon. Dural xanthogranulomas usually arise in association with familial hypercholesterolemia, with Erdheim Chester disease (ECD), and with Weber-Christian disease. Our case however, had no such associations. In this report, the authors describe the clinical, radiological and microscopic presentation of this case and the differential diagnoses of intracranial xanthogranuloma.     

Panniculitis: A summary

The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of “biological” therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Polyarteritis nodosa and Behçet's disease are the conditions that are based in the subcutaneous septa with vasculitis. Predominantly-lobular panniculitides with no vasculitis include pancreatogenic panniculitis, the panniculitis of alpha-1-antitrypsin deficiency, panniculitis associated with lupus erythematosus and dermatomyositis, subcutaneous Sweet syndrome, eosinophilic panniculitis, factitial panniculitis, cold panniculitis, panniculitis following injections of corticosteroids, lipomembranous (ischemic) panniculitis; sclerema neonatorum and subcutaneous fat necrosis of the newborn, and Rosai-Dorfman disease of the subcutis. Erythema induratum and infectious panniculitis are vasculitic and lobulocentric conditions. This article reviews the histological features of these diseases.     

Fatal pancreatic panniculitis associated with acute pancreatitis: a case report

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occurs in the setting of pancreatic diseases; these diseases include acute and chronic pancreatitis, pancreatic carcinoma, pseudocyst, and other pancreatic diseases. This malady is manifested as tender erythematous nodules on the legs, buttock, or trunk. Histopathologically, it shows the pathognomonic findings of focal subcutaneous fat necrosis and ghost-like anucleated cells with a thick shadowy wall. We herein report a case of fatal pancreatic panniculitis that was associated with acute pancreatitis in a 50-yr-old man. He presented with a 3-week history of multiple tender skin nodules, abdominal pain and distension. Laboratory and radiologic findings revealed acute pancreatitis, and skin biopsy showed pancreatic panniculitis. Despite intensive medical care, he died of multi-organ failure 3 weeks after presentation.     

Lipomembranous panniculitis: report of a case

Lipomembranous panniculitis is a peculiar type of fat necrosis and has been reported with several clinical conditions, commonly with peripheral vascular diseases. We are reporting a case of a 68-year-old woman with known liver cirrhosis and diabetes who presented with painful erythematous swelling of the right leg. Histological examination revealed microcystic changes within the subcutaneous fat. The microcysts were lined by acellular, eosinophilic membrane forming pseudopapillae and stained with periodic acid-Schiff stain. This histological feature is diagnostic of lipomembranous panniculitis. The pathologists should be aware of this entity because it aids the clinicians in investigating for associated clinical disorders.     

Mesenteric panniculitis presenting as a huge retroperitoneal mass--a case report

A rare case of mesenteric panniculitis occurring in a young patient and presenting as a huge retroperitoneal mass which was mistaken for malignancy, has been described.     

Zur Pathologie der Xanthofibrogranulomatose

Summary Two cases are described of an extensive sclerosing lesion involving the retroperitoneum, mesentery, mediastinum and epicardium which is referred as Xanthofibrogranulomatosis. The term was chosen on histological criteria: granulomatous infiltration of chronic inflammatory cells without necrosis, proliferation of spindle-shaped, lipid-laden histiocytes, aggregation of xanthomatous cells and increased production of reticulin and collagenous fibers. Furthermore the name suggests that the lesion tends to be generalized although it does not represent a true neoplasm. In nearly all cases the changes are localized in the retroperitoneal adipose tissue extending to the renal hilus and enveloping the aorta and kidneys; other organs such as adrenals and pancreas are also often surrounded. The frequent cardiac manifestations (14 of 22 cases) with sclerosis of the epicardial fat especially in the right atrioventricular region are conspicous. Moreover involvement of organs may be found, e.g. the lungs or the posterior lobe of the pituitary gland in our cases. Generally the spread of the process shows a striking relation to large blood vessels. However, the distribution lacks uniformity. The characteristic localization and histology allows the distinction of Xanthofibrogranulomatosis from other well known disorders such as Ormond's, retroperitoneal fibrosis, panniculitis (Weber-Christian disease) and histiocytosis X (Hand-Schüller-Christian). The etiology and pathogenesis of Xanthofibrogranulomatosis are uncertain. Perhaps an autoimmune mechanism involving the fat cell membrane may play a part in the genesis of this chronic inflammatory sclerosing process. As long as no rational specific therapy exists, we must interpret the xanthofibrogranulomatous lesion as a slowly but irreversibly progressing disease which is clinically comparable with a malignant tumor.

     

Persistent nodules at injection sites (aluminium granuloma) -- clinicopathological study of 14 cases with a diverse range of histological reaction patterns

AIMS: To assess and document the spectrum of histological appearances of persistent swellings which occur at injection sites following vaccination or allergen desensitization. METHODS AND RESULTS: Fourteen cases were studied. Four overlapping histological reaction patterns were evident. Ten cases showed at least focal fibrosis, fat necrosis and a mixed inflammatory cell infiltrate mainly in the subcutis, giving rise to the features of a non-specific septal and lobular panniculitis. The appearance of three cases, in addition to the non-specific panniculitis pattern, also included prominent lymphoid follicles with germinal centres and a prominent perifollicular infiltrate resembling a lymphoma (pseudolymphoma pattern). A single case mimicked lupus profundus, with a perivascular and periadnexal infiltrate in the dermis and hyaline fat necrosis. Three cases showed a predominantly palisaded histiocytic infiltrate surrounding eosinophilic necrobiosis, in a pattern closely resembling deep granuloma annulare or rheumatoid nodule. The remaining case partly showed this appearance, but in combination with panniculitis, thus demonstrating an overlap of patterns. A common feature in all 14 cases was the focal presence of histiocytes with abundant violaceous granular cytoplasm. These were shown to contain aluminium on energy dispersive X-ray microanalysis. CONCLUSION: Persistent swellings at injections sites show a variety of overlapping patterns, which mimic other conditions. Identification of characteristic histiocytes with violaceous granular cytoplasm is the key distinctive feature allowing the correct diagnosis to be reached.     

Pfeifer-Weber-Christian syndrome of the mesentery. Clinical and pathologico-anatomical aspects

A panniculitis isolated solely to the fat tissue of the mesentery is rarely encountered. Problems related to the diagnosis, therapy, and-patho-anatomical features of this polyetiological disease are presented. Based on the excellent response to antirheumatic therapy accompanied by a positive HLA B27, this case could be classified as a connective tissue disease.     

Intravascular large B-cell lymphoma presenting as cutaneous panniculitis

In this report the case of intravascular large B-cell lymphoma (angiotropic lymphoma, intravascular angioendotheliomatosis, malignant endotheliomatosis) with panniculitis features is present. The diagnosis was based on finding of typical CD20 positive tumorous cells within capillaries, veins, and small arteries in the biopsy of subcutaneous adipose tissue. An accurate and early diagnosis together with combined chemotherapy resulted in good response and remission of the disease.