Ahó H J, Forsten Y, Hopsu-Havu V K
Department of Pathology, University of Turku, Finland.
J Cutan Pathol. 1991 Oct;18(5):347-52. doi: 10.1111/j.1600-0560.1991.tb01548.x.
An asymptomatic 24-year-old woman presented with multiple discrete papules on the extensor surfaces of the hands and wrists. Light microscopy revealed focal increase in the amount of dermal fibroblasts as well as deposition of hyaluronidase-labile mucoid substance. The collagen and elastin were decreased. The changes were consistent with acral persistent papular mucinosis (APPM). In electron microscopy, the intercellular glycosaminoglycans showed small ruthenium red-positive granules and thin filaments indicating normal morphology. The fibroblastic cells, however, were conspicuously altered. Endoplasmic reticulum was dilated, cytoplasm contained large amounts of osmiophilic, concentric lysosomal structures, and there was distinct fibrous lamina in the nuclear membrane. It was concluded that the primary event in APPM probably affects the intracellular metabolism of the dermal fibroblast. The accumulation of lysosomal structures may be a distinct feature of APPM differentiating it from the other reactive cutaneous mucinoses, or it may only reflect nonspecific degeneration in a long-standing lesion.
一名24岁无症状女性，双手及手腕伸侧出现多个散在丘疹。光镜检查显示真皮成纤维细胞数量局灶性增加，以及透明质酸酶不稳定的黏液样物质沉积。胶原蛋白和弹性蛋白减少。这些改变符合肢端持久性丘疹性黏蛋白病（APPM）。电镜检查中，细胞间糖胺聚糖显示小的钌红阳性颗粒和细丝，表明形态正常。然而，成纤维细胞明显改变。内质网扩张，细胞质含有大量嗜锇性同心溶酶体结构，核膜有明显的纤维层。得出结论，APPM的主要病变可能影响真皮成纤维细胞的细胞内代谢。溶酶体结构的积累可能是APPM区别于其他反应性皮肤黏蛋白病的一个显著特征，或者它可能仅反映长期病变中的非特异性变性。
