Recent advances in understanding the etiology and treatment of systemic sclerosis (SSc) were overviewed. In vivo overexpression of connective tissue growth factor (CTGF) mRNA has been demonstrated in dermal fibroblasts in patients with SSc. Serum CTGF levels are elevated in SSc patients and are correlated with the extent of skin and lung fibrosis. Thus, CTGF appears to be the key molecule in the pathogenesis of the disease. Fetal microchimerism is another issue in the pathogenesis of SSc. Recent studies have demonstrated that fetal microchimerism was more frequent in women with SSc than in normal women. Additional investigations are needed to verify the role of microchimerism in the pathogenesis of the disease. The effects of low-dose and high-dose D-penicillamine (D-Pen) were compared in a double-blind, randomized, controlled study. High-dose D-Pen was not more effective than low-dose D-Pen, suggesting that D-Pen itself may not be effective at all. Photopheresis (extracorporeal photochemotherapy) may have limited effects, but the results of a recent crossover trial were disappointing. Low-dose relaxin was effective in a double-blind, placebocontrolled study, but high-dose relaxin was not more effective than placebo. Epoprostenol (prostaglandin I2) seems to be an effective agent for treating isolated pulmonary hypertension in patients with SSc.
Von Recklinghausen病における多発性神経線維腫は，思春期以降に多発し，整容的な点においてしばしば問題となる．今回，我々は，小型の皮膚神経線維腫に対し，無水エタノール0.1～0.3 mlを局注し，その効果を検討した．注入は，数週間に1回行い，治療開始1カ月後より小結節の縮小を認め，臨床的に満足する結果が得られた．病理組織学的には，神経線維腫細胞巣のびまん性壊死が認められた．治療後数日間の疼痛を訴えたが，経過中に改善した．外来にて簡便に施行でき，今後，切除療法に代わる有効な治療になる可能性があると思われる．