Klehr N, Heine H, Schaeg G, Nasemann T
Hautarzt. 1977 Oct;28(10):522-34.
A 44 years old male is reported who had aquired dermatochalasis and urticaria pigmentosa at the same time. This is interpreted as a subsequent phenomenon of a dermatosparaxis endoplasmatica. Clinic, histology and electron microscopy are elaborated. Systemic involvement is verified by x-rays. Histologically, predominant is the severe atrophy of the elastica, the collagenous degeneration, the presence of mastocytes, the subepidermal blister formation and the secondary xanthomatosis. The picture deviates from the common dermatosparaxis occuring in calves by the paracrystalline content of the procollagene in the endoplasmatic reticulum as verified by electron microscopy. A severe disturbance of the synthesis of collagene exists in the state of procollagene for which here the term "dermatosparaxis endoplasmatica" is suggested.
据报道,一名44岁男性同时患有获得性皮肤松弛症和色素性荨麻疹。这被解释为内质性皮肤松解症的后续现象。阐述了临床、组织学和电子显微镜检查情况。通过X射线证实存在全身受累。组织学上,主要表现为弹性组织严重萎缩、胶原变性、肥大细胞存在、表皮下疱形成和继发性黄瘤病。通过电子显微镜证实,内质网中前胶原的副晶状内容物使该病例的表现与犊牛常见的皮肤松解症有所不同。在前胶原状态下存在严重的胶原合成障碍,为此本文建议使用“内质性皮肤松解症”这一术语。