Bonerandi J J, Andrac L, Follana J, Moreau S, Aubert L
Ann Dermatol Venereol. 1980 Jan-Feb;107(1-2):51-7.
Referring to two other cases reported in the literature, the authors suggest the individualisation of a new form of cutaneous mucinosis which is characterized:--clinically, by the young age of the patient, the peculiar topography of the eruption extending to the face, neck, scalp, abdomen and thighs and the association to deep nodules of the face and the periarticular regions;--biologically by the absence of inflammation, dysglobulinemia, endocrinologic changes or bone marrow plasmocytosis;--by the acute on set of the lesions with spontaneous resolution within a few weeks. The diagnosis is based on histologic and histochemical criteria. The mucinous change occurs in the reticular dermis. Ultrastructural features are similar to those found in other dermal mucinoses. The deposit appears to be composed of an electron lucent matrix containing a micro fibrillar network, probably of polysaccharidic nature. In spite of the histochemical similarity to epithelial mucins, the biochemical composition and mode of production of the deposited substance remain to be elucidated.
参考文献中报道的另外两个病例，作者建议对一种新型皮肤粘蛋白病进行个体化研究，其特征如下：——临床方面，患者年龄较轻，皮疹的特殊分布延伸至面部、颈部、头皮、腹部和大腿，且与面部及关节周围区域的深部结节相关联；——生物学方面，无炎症、球蛋白异常血症、内分泌变化或骨髓浆细胞增多；——病变急性起病，数周内可自发消退。诊断基于组织学和组织化学标准。粘液性改变发生在网状真皮层。超微结构特征与其他皮肤粘蛋白病相似。沉积物似乎由含有微纤维网络的电子透明基质组成，可能具有多糖性质。尽管在组织化学上与上皮粘蛋白相似，但沉积物质的生化组成和产生方式仍有待阐明。
