A Case of Dermatomyositis Coexisting with Both Anti-Mi-2 and Anti-NXP-2 Antibodies.

Myositis-specific autoantibodies (MSAs) including anti-Mi-2 and anti-nuclear matrix protein 2 (NXP-2) antibodies have been detected in the patients with dermatomyositis (DM), and are useful tools for identifying clinical subsets of DM. MSAs are exclusively found in DM patients. Anti-Mi-2 antibody-positive DM patients show the typical skin lesions and myositis and are rarely associated with internal malignancy and interstitial lung disease (ILD). On the other hand, adult DM patients with anti-NXP-2 antibody often show calcinosis and internal malignancy, but rarely ILD. In addition, anti-NXP-2 antibody-positive DM patients have severe phenotype with myalgia, peripheral edema, and significant dysphagia, but with mild skin lesions. Herein, we report a rare case of classic DM coexisting with both anti-Mi-2 and anti-NXP-2 antibodies, clinically, without ILD or internal malignancy. Our patient had typical skin manifestations, muscle weakness, muscle pain, and general fatigue without calcinosis, peripheral edema, or dysphagia. Thus, the clinical phenotype was similar to anti-Mi-2 antibody-positive DM.

Drug-induced Pressure Ulcers in a Middle-aged Patient with Early-stage Parkinson's Disease.

Drug-induced pressure ulcer (DIPU), which is a newly recognized adverse drug reaction, is associated with the administration of psychiatric drugs in geriatric patients with dementia. The notification of the causative drugs is crucial to the treatment of DIPU. We herein report the case of a 56-year-old woman with early-stage Parkinson's disease who developed DIPUs after starting olanzapine treatment for depressive symptoms. Our findings illustrate that if an akinetic patient with pressure ulcers is encountered, the patient's medication should be reviewed by a multidisciplinary team, to evaluate whether the development of the pressure ulcer is drug-related, regardless of the patient's age.

Three siblings with systemic lupus erythematosus.

We present the cases of three siblings with systemic lupus erythematosus (SLE). The diagnosis was made when the sisters were of age 21, 25 and 28 years. They shared some clinical features, including typical facial rash, photosensitivity and Raynaud's phenomenon, and tested positive for antinuclear antibodies. However, their symptoms and clinical courses varied. Human leukocyte antigen (HLA) typing revealed that DR4 and A2 were present in all three sisters, while HLA type A11, B35 and B54 were each found in two of the three sisters. The two elder sisters developed lupus glomerulonephritis 8 and 11 years after the onset of SLE. It is suggested that there are genes responsible for the onset of the disease and also unknown regulatory genes other than HLA result in different phenotypes.

Catabolism of pemphigus foliaceus autoantibodies by high-dose IVIg therapy.

High-dose intravenous immunoglobulin (HD-IVIg) has several distinguishing therapeutic characteristics. However, a certain number of pemphigus cases have been experienced, which did not respond to HD-IVIg. This is the first case report that the serum level of anti-desmoglein (Dsg) 1 antibody rebounded, critically associated with IgG serum level. We describe a patient with pemphigus foliaceus (PF), unresponsive to oral prednisolone followed by pulse therapy and double-filtration plasmapheresis, in whom clinical remission was induced by 4 courses of HD-IVIg. Anti-Dsg1 antibody levels, serum IgG and disease activity were monitored. Anti-Dsg1 antibody titers rapidly decreased after IVIg treatment when total IgG levels were high; however, the serum level of anti-Dsg1 antibody rebounded as the total IgG level returned to normal. The levels of anti-Dsg1 antibody were decreased for an average of 13.7 days after treatment. The therapeutic effect of IVIg treatment was associated with an increased serum level of total IgG. IVIg therapy reduced the titers of autoantibody by accelerating the catabolism of immunoglobulin induced by high IgG serum levels. IVIg itself appears to accelerate IgG degradation rather than suppress IgG production. Sufficient suppression of antibody production is critical for successful treatment with IVIg.