Berges W, Merk H, Günther D, Goerz G, Wienbeck M
Dtsch Med Wochenschr. 1981 Jun 5;106(23):747-51. doi: 10.1055/s-2008-1070394.
Scleroedema adultorum Buschke is characterized by progressive hardening of the skin. In contrast to scleroderma the hardening occurs in the skin of the trunk while extremities remain largely free. Internal organs are said not to be involved in scleroedema adultorum Buschke. The full picture of the persistent form of scleroedema adultorum Buschke was observed in two patients. One patient complained of increasing dysphagia with regurgitation and aspiration. Manometry and X-ray cinematographic investigation showed inappropriate relaxation of the upper oesophageal sphincter. In the other patient who had not previously had swallowed difficulties manometry showed achalasia of the upper oesophageal sphincter. The functional disturbances of the upper oesophagus indicate the possibility of an involvement of internal organs in scleroedema adultorum Buschke. However, proof of an aetiological connection between disturbances of oesophageal motility and skin disease requires systematic investigations in a larger group of patients.
成人硬肿病(Buschke型)的特征是皮肤进行性硬化。与硬皮病不同,硬化发生在躯干皮肤,而四肢基本不受影响。据说成人硬肿病(Buschke型)不累及内脏器官。在两名患者中观察到了成人硬肿病(Buschke型)持续型的全貌。一名患者主诉吞咽困难加重伴反流和误吸。测压和X线电影造影检查显示食管上括约肌松弛异常。在另一名既往无吞咽困难的患者中,测压显示食管上括约肌失弛缓症。食管上段的功能障碍表明成人硬肿病(Buschke型)可能累及内脏器官。然而,要证明食管动力障碍与皮肤病之间存在病因学联系,需要对更多患者进行系统研究。