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

Abstract

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.

Lipoatrophic Connective Tissue Panniculitis

“…In these patients, systemic disorders including diabetes mellitus, Hashimoto’s thyroiditis, juvenile rheumatoid arthritis, and metabolic disorders (hypertriglyceridemia and hyperglycemia with insulin resistance) were associated, supporting the hypothesis of an autoimmune pathogenesis. Other reports mentioned the association of connective tissue panniculitis in children with vitiligo, Hashimoto’s disease, and autoimmune hepatitis (6,7). Finally, lipoatrophic panniculitis has been reported in a child presenting with persistent circulating antibody (rheumatoid factor and anti‐DNAs) and partial IgA deficiency (8).…”

Childhood Weber–Christian disease: clinical investigation and virus detection

“…The study of Iwasaki et al implicated that the T‐cell immune responses in the pathogenesis of WCD because the patient had extremely elevated levels of interferon‐gamma and soluble interleukin‐2 receptor (25). Moreover, several previous studies showed that some forms of panniculitis may be associated with virus infection, such as hepatitis virus and human T‐cell lymphotropic virus, which implied that the WCD may be an adipose tissue response to a variety of immunological stimuli, including virus (7–9,26). However, no antigens of EBV, CMV, HSV1 and HSV2 were found from the biopsy tissue section, and no significant titers of viral antibodies were detected from blood samples.…”

Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices

“…Some cases of variceal bleeding due to extrahepatic portal hypertension from mass effect of fat necrosis and liver fibrosis have also been described. 25,26 The most common histopathologic finding reported is the different degree of macro-vesicular steatosis (sometimes contained foam cells), 19 with 1,3,4,6 or without 5 hepatocyte necrosis and mononuclear cells infiltration. 6 In some cases, the presence of Mallory bodies has been evidenced.…”

“…2 Several cases with hepatomegaly or splenomegaly and changes in liver function have been published. 3-7 Systemic symptoms and signs such as relapsing fever episodes, fatigue, and polyarthralgia are also frequent. On the contrary, hematological abnormalities and visceral involvement of organs including lungs, heart, intestines, spleen, kidneys, and adrenal gland are rarely described.…”