Kiyohiro Tsutsui

Clinical Correlations With Dermatomyositis-Specific Autoantibodies in Adult Japanese Patients With Dermatomyositis: A Multicenter Cross-sectional Study

OBJECTIVE To clarify the association of clinical and prognostic features with dermatomyositis (DM)-specific autoantibodies (Abs) in adult Japanese patients with DM. DESIGN Retrospective study. SETTING Kanazawa University Graduate School of Medical Science Department of Dermatology and collaborating medical centers. Patients A total of 376 consecutive adult Japanese patients with DM who visited our hospital or collaborating medical centers between 2003 and 2008. MAIN OUTCOME MEASURES Clinical and laboratory characteristics of adult Japanese patients with DM and DM-specific Abs that include Abs against Mi-2, 155/140, and CADM-140. RESULTS In patients with DM, anti-Mi-2, anti-155/140, and anti-CADM-140 were detected in 9 (2%), 25 (7%), and 43 (11%), respectively. These DM-specific Abs were mutually exclusive and were detected in none of 34 patients with polymyositis, 326 with systemic sclerosis, and 97 with systemic lupus erythematosus. Anti-Mi-2 was associated with classical DM without interstitial lung disease or malignancy, whereas anti-155/140 was associated with malignancy. Patients with anti-CADM-140 frequently had clinically amyopathic DM and rapidly progressive interstitial lung disease. Cumulative survival rates were more favorable in patients with anti-Mi-2 compared with those with anti-155/140 or anti-CADM-140 (P < .01 for both comparisons). Nearly all deaths occurred within 1 year after diagnosis in patients with anti-CADM-140. Conclusion Dermatomyositis-specific Abs define clinically distinct subsets and are useful for predicting clinical outcomes in patients with DM.

Annular Elastolytic Giant Cell Granuloma: Response to Cyclosporin A

A case of annular elastolytic giant cell granuloma with a good response to cyclosporin A is reported. A 62‐year‐old man developed multiple annular patches on the trunk with elevated and indurated borders. Biopsy specimens from the border showed granulomatous inflammation in the mid dermis with phagocytosis of elastic fibers by giant cells. Biopsy specimens from the center showed dense collagen formation without inflammation. Immunological investigation of perivascular infiltrating cells in the lesions revealed a predominance of CD4+ cells over CD8+ cells. Our case showed a good response to cyclosporin A (5 mg/kg/day) for eight weeks. There were no adverse effects and no recurrences for one month after discontinuation of cyclosporin A.

Successful Treatment of Livedo Vasculitis with Beraprost Sodium: A Possible Mechanism of Thrombomodulin Upregulation

BACKGROUND Livedo vasculitis is thought to be a thrombogenic disorder. We demonstrated that thrombomodulin (TM) expression on the endothelial cells in livedo vasculitis was markedly reduced, while blood tests of coagulation and fibrinolytic activities were within the normal range. OBJECTIVE Since prostacyclin (PGI2) upregulates the TM expression of endothelial cells, we tried a PGI2 analogue for the treatment of livedo vasculitis. METHOD Four patients with livedo vasculitis were started on a regimen of beraprost sodium (120 micrograms daily). Additional intake of low-dose aspirin was combinated with a maintenance dose of beraprost sodium (60 micrograms daily). RESULT All 4 patients experienced a clinical improvement with a combination therapy with beraprost sodium and low-dose aspirin. CONCLUSION We propose that PGI2 analogue therapy is useful for the treatment of livedo vasculitis, since the drug upregulates TM expression in this disorder.

Widespread pruritic plaques in a patient with subacute cutaneous lupus erythematosus and hypocomplementemia: Response to dapsone therapy

We describe a patient with subacute cutaneous lupus erythematosus, widespread pruritic papulosquamous plaques, and hypocomplementemia. Skin biopsy specimens revealed liquefaction degeneration and colloid bodies and dyskeratotic cells in basal and suprabasal layers. An immunofluorescence study revealed deposits of IgG, IgM, and C3 at the dermalepidermal junction in a bandlike pattern, and particulate IgG deposition in the basal and suprabasal layers. Treatment with prednisolone (15 mg/day), chloroquine phosphate (200 mg/ day), cyclosporine (5 mg/kg daily), and gold (10 mg/day) failed to reduce pruritic plaque formation, and pulse methylprednisolone therapy led to only a transient remission. Clinical exacerbations correlated with a decrease in complement levels. The disease was successfully controlled with dapsone (75 mg/day) and prednisolone (25 mg/day).