We have previously shown that the serum TARC/CCL17 level reflects the disease activity of atopic dermatitis (AD). In this study we further examined this phenomenon in a larger number of patients and confirmed it. Furthermore, in a longitudinal study of patients with AD, serum TARC/CCL17 levels was examined and compared to other various laboratory markers such as serum LDH, serum IgE, and eosinophil number in peripheral blood. We found that serum TARC/CCL17 levels reflected the disease activity of AD most sensitively than the other laboratory markers. These results indicate that serum TARC/CCL17 levels can be used as a novel laboratory marker to reflect the disease activity of AD.
We retrospectively examined 614 pregnant women with cutaneous manifestations to determine the prevalence of cutaneous diseases during pregnancy. The 614 patients visited the dermatology clinic at Seibo Hospital between April of 1998 and March of 2002. Their ages ranged from 17 to 42 (ave. 29.6 y-o); 456 of them (74.3%) were primiparous. The diseases of patients were divided into 5 groups : eczema or dermatitis (321 patients), prurigo or urticaria (90 patients), infectious diseases (132 patients), cutaneous benign tumors (41 patients), and others (84 patients). When categorized by the relationship of the cutaneous disease to pregnancy, 0.3% of the patients had pregnancy specific dermatoses, 9.6% had pregnancy-induced skin changes, 6.6% had exacerbations or improvements of existing skin diseases during pregnancy, and 84.1% had common skin diseases unrelated to pregnancy. Thus, the vast majority (84.2%) of cutaneous diseases during pregnancy were, in fact, unrelated to pregnancy (eczema was particularly common.) We further discuss the possible treatment options for pregnant patients with skin diseases during pregnancy.
We report two patients, a 59-year-old female and a 21-year-old female with systemic sclerosis (SSc) who demonstrated myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA). Both patients developed crescentic glomerulonephritis and mononeuritis multiplex, respectively, nine years after the onset of SSc. We diagnosed these patients as having an overlap of SSc and MPO-ANCA-related vasculitis. A combination of oral administration of prednisolone and intravenous cyclophosphamide improved their vasculitic symptoms. We determined the incidence of ANCA-related vasculitis in SSc patients at our hospital. Of the 21 SSc patients examined, only the two patients described here were MPO-ANCA positive. A literature review demonstrated that the features of Japanease SSc patients who produce MPO-ANCA included a high incidence of positive anti-topoisomerase I antibody (71%) and a long lag phase between the onset of SSc and the development of MPO-ANCA (average 10 years, 0.5～30 years). Although the development of MPO-ANCA-related vasculitis is extremely rare in SSc patients, it is clinically of great importance. Acute progressive glomerulonephritis as well as pulmonary hemorrhage caused by ANCA-related vasculitis can determine the prognosis of such patients. If clinically evident vasculitis occurs in an SSc patient, particularly a patient with positive anti-topoisomerase I antibody and a long lag phase from the onset of SSc, prompt diagnosis of ANCA-related vasculitis and the early initiation of immunosuppressive therapy, particularly in the case of life-threatening conditions, can be determinative factors for a good outcome.
It is well known that immunodeficient patients suffering from HIV infection, blood diseases or collagen diseases, tend to have the complication of toxoplasmosis. We reported two male patients complicated by collagen disease and toxoplasmosis. In Case 1, a 49-year-old male patient with systemic lupus erythematosus (SLE)- scleroderma (SSc) overlap syndrome developed muscle symptoms similar to those from dermatomyositis. We finally diagnosed him as having toxoplasmosis, because of the high titer of anti-toxoplasma antibody and of the rapid disappearance of the symptoms after treatment with anti-toxoplasma agents. In Case 2, a 56-year-old male patient with dermatomyositis, showed a visual defect caused by toxoplasmosis. Many previous reports have shown that titers of anti-toxoplasma antibody in patients with PM/DM were significantly higher than in those with other diseases. However, our assessment of the titers in 190 patients with collagen disease showed no significant differences among various collagen diseases.
We reported a rare case of diffuse cutaneous systemic sclerosis (SSc) with catastrophic antiphospholipid syndrome (CAPS). A 70-year-old woman with SSc visited our hospital in November 7, 2002, and she also had extreme hypertension. Despite treatments with an inhibitor of angiotensin converting enzyme (ACEI) to lower her blood pressure, her renal function gradually deteriorated until finally she had to receive dialysis treatment. About two years after the first visit, she complained of general fatigue and severe cough ; pneumonia and heart failure were suspected. After a brief improvement with treatment, she suddenly suffered from circulation failure of the limbs and drastic disturbance of consciousness. Because of her positive lupus anticoagulant (LA) and of thrombosis detected in many internal organs, we diagnosed her with CAPS. We tried anticoagulant therapy and plasma exchange, but she died without improving. It is well known that a severe, rapidly progressive form characterized by clinical involvement of multiple different organ systems with histopathological evidence of small and large vessel occlusion is termed CAPS, and that patients with systemic lupus erythematosus (SLE) often suffer from CAPS. Some previous studies have indicated that more than 50% of SSc patients have LA. However, it is still not clear whether or not the patients with SSc have a high incidence of APS or CAPS, as in this case of SLE.