Weber-Christian Disease with Visceral Involvement

Systemic Form of Weber-Christian Disease with Hepatic Enlargement

“…In rare instances, systemic involvement may appear before the skin eruption, which leads to diagnostic difficulties, but visceral involvement is rarely so extensive as to result in death [4], In our case the liver was enlarged and there were no signs of other internal organ involvement. O ran and C o c h ra n e [4] described a case of a 63-year-old housewife with hepatic enlarge ment and microscopically normal liver architecture, but with a moderate degree of fatty degeneration without necrosis, fibrosis or cellular infiltrate.…”

“…However, in a few cases various viscera and other organs may be involved in the same pathological process and may result in the patient's death. Other cases with considerable hepatic enlargement were also described by other authors [3,4], It is well known that any toxic or infective process can produce slight fatty changes in the liver. However, it appears that gross fatty metamorphosis can take place in the liver as well as in other organs and give rise to conside rable enlargement.…”

“…It has been suggested that an allergy to bacterial, chemical or even viral substances produces metamorphosis in the fat. which then acts as a foreign body in the tissues and gives rise to an inflammatory reaction [4]. According to R ook et al [1] the most widely held view assumes that the primary abnormality is an allergic vasculitis affecting the vessels of the subcutis, with secondary fat cell necrosis.…”

“…O ran and C o c h ra n e [4] described a case of a 63-year-old housewife with hepatic enlarge ment and microscopically normal liver architecture, but with a moderate degree of fatty degeneration without necrosis, fibrosis or cellular infiltrate. A liver biopsy of our case showed a more prominent infiltration and degenera tion of hepatocytes.…”

Crescentic Glomerulonephritisin a Patient with Relapsing Nodular Pannicul itis

“…The association of vasculitis with panniculitis has been recognized for many years and is seen in erythema induratum, chilblains and periarteritis nodosa as well as in some patients who present with relapsing febrile nodular nonsuppurative panniculitis. This latter condition, also known as Weber-Christian disease, may sometimes only involve the skin, but can also lead to perivisceral involvement with systemic illness and death [7]. In patients with fatal panniculitis, although multiorgan involvement is common, it is rare for the kidneys to be affected [8].…”