De Leo G, Losacco T, Punzo C, Lavacca M, Pitzalis M V, De Tommaso C, Posca A
Istituto di Semeiotica Chirurgica, Università degli Studi di Bari.
G Chir. 1994 Mar;15(3):97-102.
The Authors report an exceptional case of Atrophic Malignant Papulosis (P.A.M.) or Degos disease, presenting as acute abdomen with ileal and multiple colic perforations. The disease is due to an occlusive, progressive, thrombotic vascular pathology affecting different parts of the body and may bring to death. Usually this rare disease presents with typical multiple papulous skin lesions, followed by a systemic involvement of the digestive, nervous, and visive tracts. Peritonitis and cerebral infarction are the most frequent causes of death.
作者报告了一例萎缩性恶性丘疹病（P.A.M.）或德戈斯病的罕见病例，表现为急性腹痛伴回肠和多处结肠穿孔。该病是由一种闭塞性、进行性、血栓性血管病变引起，影响身体不同部位，可能导致死亡。通常这种罕见疾病表现为典型的多发性丘疹性皮肤病变，随后出现消化道、神经和视觉系统的全身性受累。腹膜炎和脑梗死是最常见的死亡原因。
