A case of Weber-Christian disease accompanied by a nasal symptom (a clinicopathologic case report)

A rare case of fatal Weber-Christian disease is reported. The initial presenting signs were unilateral nasal obstruction due to hyperplasia of the ulcerated turbinate, swollen cheeks and eyelids accompanied by recurrent febrile episodes. The nasal lesion showed marked inflammatory granulation tissue with necrosis and there were 'atypical' reticulohistiocytic cells. Subsequently the patient developed widespread nodular nonsuppurative panniculitis of the left thigh. The patient's general condition worsened in spite of cytotoxic and steroid treatment and he died of acute peritonitis from a perforated duodenal ulcer complicating the treatment. Microscopy showed that some atypical cells were present not only in the nasal lesion but also in the lesions of the skin and in the sinusoids of the liver and in the spleen. Weber-Christian disease secondary to midline granuloma or malignant histiocytosis has not previously been reported.