Ceuterick C, Martin J J
Department of Neuropathology, Born-Bunge Foundation, University of Antwerp, Belgium.
Pathol Res Pract. 1993 May;189(4):384-6. doi: 10.1016/S0344-0338(11)80323-7.
Evidence for storage in eccrine sweat glands from skin biopsies of 7 patients with Krabbe globoid cell leukodystrophy is demonstrated. Clear cells showed characteristic free or intravacuolar inclusions containing needle-like and splinter-shaped structures with sometimes clearly outlined paracrystalline-like geometric configurations. These inclusions were very similar to those observed in the characteristic globoid cells found in the central nervous system and in Schwann cells of cutaneous, conjunctival and peripheral nerves. This finding suggests that the storage is not restricted to the nervous system. The diagnostic value of skin biopsy in Krabbe globoid cell leukodystrophy is emphasized.
对7例克拉伯球状细胞脑白质营养不良患者的皮肤活检显示，有证据表明其在小汗腺中存在储存现象。透明细胞显示出特征性的游离或泡内包涵体，其中含有针状和碎片状结构，有时还有轮廓清晰的类副晶状几何构型。这些包涵体与在中枢神经系统以及皮肤、结膜和周围神经的施万细胞中发现的特征性球状细胞中观察到的包涵体非常相似。这一发现表明，储存现象并不局限于神经系统。强调了皮肤活检在克拉伯球状细胞脑白质营养不良中的诊断价值。
