The number of lymphatic vessels has been reported to be lower in the lesional skin of systemic sclerosis (SSc) patients than in the skin of healthy controls. We examined the number of lymphatic vessels in the papillary and reticular dermis of forearm skin of SSc patients (41 cases) using immunostaining for D2-40, a selective marker for lymphatic endothelium. Lymphatic vessels in the dermis were significantly decreased in number in the patients with severe dermal fibrosis. The number of lymphatic vessels was also significantly decreased in patients with anti-topoisomerase I antibody-positive diffuse cutaneous SSc compared with that of patients with anti-centromere antibody-positive limited cutaneous SSc (P<0.05). The differences were significant, especially in the reticular dermis, suggesting that lymphangiopathy at the level of collector vessels may have a diverse pathogenetic role in the progression of SSc.
Immunosuppressive therapy is one of the options available for the initial treatment of severe pemphigus in the control phase. In this retrospective study, the add-on effect of an immunosuppressant as an adjuvant therapy to systemic corticosteroid therapy from the consolidation phase to the maintenance phase was examined. The cases of 16 of the 35 pemphigus patients in the consolidation phase or in the maintenance phase treated with an immunosuppressant at Okayama University Hospital between 2012 and 2014 were analyzed. Outcomes were evaluated based on the degree of changes in the Pemphigus Disease Area Index, serum autoantibody levels, and dose of corticosteroid. When at least one of these evaluation points declined, the treatment was judged as effective. Thirteen patients received azathioprine, four received cyclosporine, and eight received mizoribine in combination with systemic corticosteroid therapy during the clinical course. Effective add-on therapy was observed in 92%, 100%, and 63% of the patients treated with azathioprine, cyclosporine and mizoribine, respectively. Progression of disease activity after discontinuation of the immunosuppressant was observed in 46%, 50%, and 25% of these groups. These data indicate that the addition of either azathioprine or cyclosporine to corticosteroid therapy is a viable treatment option in pemphigus patients who are unresponsive to corticosteroids during the consolidation to maintenance phases.