[Case report of effective intravenous immunoglobulin for lower limbs pain in steroid-resistant eosinophilic granulomatosis with polyangiitis].

We report a 58-year-old woman with bronchial asthma. The onset of the disease was marked by numbness in the right lower extremity, for which she was hospitalized 10 days later. The patient presented with sensory impairment and muscle weakness in the distal regions of both lower limbs, acute pain, purpura, and a leukocyte count of 2.4 × 10(4)/µl (59.2% eosinophils). Nerve conduction tests revealed a decrease in the amplitude of the compound muscle action potential in all 4 extremities. Skin biopsy results led to the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Steroid pulse therapy and oral steroid therapy were initiated but did not resolve the acute pain or numbness. However, intravenous immunoglobulin (IVIg) was administered at day 28 after the beginning of the steroid treatment, and the pain started to improve immediately afterward. In some cases, IVIg can be effective in the treatment of intense pain in peripheral neuropathy associated with steroid-resistant EGPA.

Severe gastrointestinal involvement in paraneoplastic pemphigus.

Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease, associated with neoplasia, which has characteristic clinical, histological and immunological features. While respiratory epithelial involvement has been described in several cases, lesions in the colon epithelium have never been reported. We describe a 57-year-old Japanese woman with PNP who had many aphthae-like erosions on the colon epithelium, in addition to typical mucocutaneous PNP lesions. The intestinal erosions had histological features similar to those of PNP and linear deposition of complement, but not IgG, was observed along the colon epithelial basement membrane.