Red painful nodules in a hospitalized patient.

Was the patient's treatment, or his underlying condition, to blame?

[Weber-Christian disease].

In pursuance of the Federal law "On the fundamentals of health protection in RF", the Russian Ministry of Health approved the list of so-called orphan diseases, i.e. rare pathologies of great medical and social significance due to their life-threatening nature and immense burden on health budget. Weber-Christian disease is a chronic recurrent orphan diseases characterized by formation of painful dense nodes in subcutaneous fat free from suppuration and accompanied by episodic temperature changes, chill, muscular pain and sometimes damage to internal organs. The disease recurs at irregular intervals.

A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report.

BACKGROUND: Pfeifer-Weber-Christian disease (PWCD) is a rare inflammatory disorder of the subcutaneous fatty tissue. The diagnosis and therapy of this rare type of panniculitis is still controversial and will be discussed in this article. CASE PRESENTATION: We here report the rare case of a 64-year old male patient, with PWCD. The patient suffered from rheumatoid arthritis for several years, but then developed relapsing fever and recently occurring painful subcutaneous nodules predominantly at the inner part of his left upper limb with no signs of synovitis. Finally, a biopsy from one of the nodules revealed lobular panniculitis with mixed cell infiltrate, which was conformable only with PWCD, after excluding several differential diagnoses. In our patient PWCD developed despite immunosuppressive therapy with steroids and different disease modifying drugs, which the patient received to treat his underlying rheumatoid arthritis. However, when DMARD therapy was switched to Ciclosporin A the patient's symptoms resolved. CONCLUSION: Our observation supports the hypothesis that T cells are involved in the pathogenesis of PWCD. Thus, T cell modifying drugs should be primarily used to treat patients with this rare disorder.

Efficacy of cyclosporine A treatment in relapsing febrile lobular panniculitis associated with small vessel vasculitis.

Weber-Christian Disease (WCD), also known as relapsing febrile lobular non-suppurative panniculitis, is a rare condition characterized by recurrent subcutaneous inflammatory nodules in the adipose tissue in addition to fever, malaise and other systemic manifestations such as polyarthralgia and polymyalgia. The association with small vessel vasculitis has been rarely reported. We report here an unusual case of WCD associated with small vessels vasculitis also describing the efficacy of Cyclosporin A treatment.

Idiopathic lobular panniculitis (Weber Christian disease): a case report.

Weber Christian disease, an idiopathic relapsing febrile subcutaneous and visceral panniculitis is a rare disease in children. We report a case of Weber Christian disease for he first time from Kanti Children's Hospital.

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

Recognizing Weber-Christian disease.

The eponym Weber-Christian Disease (WCD) defines a chronic disorder characterized by relapsing febrile episodes and panniculitis. Systemic manifestations due to visceral involvement may be present. WCD is associated with no identifiable cause, although chronic panniculitis may be due to definable underlying disorders. A variety of distinctive disease entities, such as systemic lupus erythematosus (SLE), pancreatic disease, alpha-I-antitrypsin disease, lymphoproliferative neoplasia, infections, or trauma are associated with chronic panniculitis. The accurate diagnosis of panniculitis requires an adequate deep skin biopsy showing inflammation of the subcutaneous layers. We describe a white woman with fever and recurrent episodes of painful nodules of the lower extremities, excisional biopsy of which confirmed panniculitis. The febrile episodes and skin lesions responded dramatically with the use of oral corticosteroids.

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.

Omental panniculitis: an unusual cause of acute appendiceal syndrome.

We report the case of a 41-year-old patient with no history of abdominal symptoms who presented with an acute painful syndrome of the right flank and iliac fossa suggesting acute appendicitis. The diagnosis of panniculitis of the great omentum was suggested preoperatively by the particular aspect of omental fat observed by abdominal sonography and computed tomography. A subvoval omentum resection was performed and the outcome was uneventful.

Cytophagic panniculitis.

A 60 year old woman developed generalised erythema nodosum-like lesions together with hectic fever, lymphadenopathy, and hepatitis. Biopsies revealed lobular panniculitis with a benign histiocytic infiltrate and prominent phagocytosis in subcutaneous sites, lymph nodes, and bone marrow. All immunological, serological, and culture studies were negative, apart from throat isolation of herpes simplex. The patient responded to high-dose corticosteroids. The case illustrates the differential diagnosis of lobular panniculitis, with histiocytic infiltrate and cytophagocytosis. These combined features are consistent with the recently described syndromes of cytophagic panniculitis and virus-associated hemophagocytic syndrome.

Sclerosing mesenteritis. Response to cyclophosphamide.

We present two patients with an aggressive form of sclerosing mesenteritis characterized by a progressive, life-threatening course, prominent retroperitoneal disease, and tubuloreticular structures in one case, an ultrastructural feature associated with autoimmune and cyclophosphamide-responsive diseases. In both patients, aggressive immunosuppressive medical therapy with cyclophosphamide resulted in prompt, dramatic improvement, without recurrence. When the diagnosis of sclerosing mesenteritis is established, we recommend early aggressive medical therapy with cyclophosphamide, particularly when tubuloreticular structures are present.

Weber-Christian disease. Analysis of 15 cases and review of the literature.

We report 15 patients encountered over 13 years who presented with inflammation of subcutaneous fat and were given clinical and pathologic diagnoses of Weber--Christian disease (WCD). Prominent clinical features included female predominance, lower extremity nodules, fevers, arthritis/arthralgias, and myalgias. Notable laboratory features were elevated erythrocyte sedimentation rate, anemia, leukopenia, and hypocomplementemia, frequently with circulating 7S IgM or immune complexes at times of active symptoms. Histologic findings were lobular--together with frequent septal--panniculitis, fat-laden macrophages, variable cellular infiltrates, necrosis, and occasional vasculitis. Follow-up revealed the death of 2 patients and disease stabilization or improvement in 13 patients. Six patients developed features of other diseases (factitial disease, erythema nodosum, acute myelogenous leukemia, rheumatoid arthritis, systemic lupus erythematosus, and sarcoid) and a seventh may have had erythema induratum. We suggest that classic WCD, as originally described, reflects an increasingly recognized spectrum of panniculitides. These are syndromes of diverse etiology that share many clinical, inflammatory, and immunologic features.

Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis.

We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.

Hemorrhagic diathesis in Weber-Christian disease.

A case of a 21-year-old woman with Weber-Christian disease, associated with consumption coagulopathy and adequately controlled over the past year or more by corticosteroid and heparin therapy, has been described. Through the study on this case, it was concluded that a hemorrhagic diathesis complicating Weber-Christian disease, at least in advanced cases, was characterized by consumption coagulopathy associated with enhanced blood coagulability which arises from hyperlipemia due to abnormal lipid metabolism and further, that the consumption coagulopathy was modified by diminished synthesis of clotting factors and activation of the fibrinolytic system due to the secondary hepatic impairment. It might also be likely that systemic vascular changes contribute to the activation of the clotting system as a trigger of clotting factor consumption. The complex pathophysiology of a hemorrhagic diathesis in this disease seemed to be brought about by a complex combination of such a variety of factors. In addition, decreased erythrocyte sedimentation rate observed characteristically during hemorrhage might probably have a direct correlation with hypofibrinogenemia due to consumption coagulopathy in advanced cases.