IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology

Sclerosing mesenteritis is a rare inflammatory and fibrosing disorder of unknown etiology, while IgG4-related disease (IgG4-RD) consists of mass-forming, fibroinflammatory lesions characterized by high serum IgG4 levels and tissue infiltration of many IgG4-positive plasma cells; obliterative phlebitis is common. This report describes a case of sclerosing mesenteritis that was considered a manifestation of IgG4-RD. A 53-year-old man underwent right hemicolectomy because of an ileocecal mass that did not improve with conservative therapy. The ill-defined fibroinflammatory lesion extended in the mesentery with storiform fibrosis, obliterative phlebitis, and infiltration of many IgG4-positive plasma cells. The ratio of IgG4-positive/IgG-positive cells was 64%, and the ratio of forkhead box protein 3 (FOXP3)-positive/CD4-positive cells was elevated (13%). It is likely that at least some cases of sclerosing mesenteritis are a manifestation of IgG4-RD. It is important to investigate this relationship because steroid therapy may benefit such cases.     

Recent advances in cytokines in cutaneous and systemic lupus erythematosus

Lupus erythematosus (LE) includes a broad spectrum of diseases from a cutaneous‐limited type to a systemic type. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease which affects multiple organs. Cutaneous lupus erythematosus (CLE) includes skin symptoms seen in SLE and cutaneous‐limited LE. Although immune abnormalities, as well as heritable, hormonal and environmental factors, are involved in the pathology of LE, the actual pathogenesis is still unclear. Recently, the involvement of various cytokines has been shown in the pathogenesis of LE. Moreover, some trials with biological agents targeted specific cytokines are also ongoing for SLE. In this article, we review the contributions of major cytokines such as interferon, tumor necrosis factor‐α and interleukin‐18 to LE, especially SLE and CLE.     

Endotoxin shock due to Vibrio vulnificus infection

A 54-year-old man with hepatitis C fell into shock with symptoms similar to enterocolitis after ingesting an undercooked barbecued mackerel. Most of his eruptions developed into annular erythema with small vesicles. He had taken high dose corticosteroids with intravenous cefotiam. His eruptions improved, but his shock state was exacerbated on Day 2. Treatment for endotoxin shock was initiated using piperacillin, intravenous immunoglobulin (IVIg), and hemoperfusion with Polymyxin B immobilized fiber (PMX-F), which resulted in shock reversal. The serum IL-6 value was 118,000 pg/mL on admission, and decreased to 2040 pg/mL on Day 3. On Day 6, the results from the culture of skin biopsy specimens showed the diagnosis as Vibrio vulnificus septic shock. Debridement was not needed, which is thought to be essential to Vibrio vulnificus sepsis. The changes in the serum IL-6 levels demonstrated that hemoperfusion with PMX-F and IVIg therapy was practical for Vibrio vulnificus septic shock.     

Interlamellar Flap Edema Due to Steroid-Induced Ocular Hypertension After Laser In Situ Keratomileusis

Background

Several recent studies have reported post-laser in situ keratomileusis (LASIK) complications related to a steroid-induced increase in intraocular pressure, including interface fluid and elevated intraocular pressure-induced interlamellar stromal keratitis.

Cases

We examined two cases of interlamellar flap edema due to steroid-induced ocular hypertension after uneventful laser in situ keratomileusis.

Observations

Oral acetazolamide and discontinuance of topical steroids were effective for treating interlamellar stromal edema in both cases.

Conclusion

A rise in intraocular pressure should be considered a cause of interlamellar stromal edema after LASIK.?Jpn J Ophthalmol 2007;51:228–230 © Japanese Ophthalmological Society 2007