Kim J H, Yim H, Kang W H
Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.
J Korean Med Sci. 2000 Aug;15(4):478-81. doi: 10.3346/jkms.2000.15.4.478.
Disseminated superficial porokeratosis (DSP) is a rare cause of secondary cutaneous amyloidosis. An 83-year-old male patient showed an increase in both size and number of DSP lesions after contracting pulmonary tuberculosis. The DSP lesions of the patient consisted of numerous annular eruptions on both sun-exposed and sun-protected areas, which occurred over a period of 20 years. Multiple skin biopsies were taken from normal or lesional/sun-exposed or sun-protected skin samples. Histopathologic examination included routine H&E stains, Congo red stains, thioflavin-T stains and anticytokeratin antibodies (AE1, AE3). And the results were as follows; 1) Positive staining with Congo red and thioflavin-T indicated an amyloid nature for the deposits, 2) confinement of the amyloid deposition just below the lesional epidermis (while sparing the neighboring uninvolved or distant normal skin) indicated some role of the lesional epidermis, and 3) positive staining with AE3 further indicated an epidermal origin-type II epithelial keratin-of the amyloid. We present a case of DSP with a local amyloid deposit, characterized by association of positive familial background, severe pruritus and pulmonary tuberculosis.
播散性浅表性光化性角化病(DSP)是继发性皮肤淀粉样变的罕见病因。一名83岁男性患者在患肺结核后,DSP皮损的大小和数量均增加。该患者的DSP皮损由暴露于阳光和未暴露于阳光区域的大量环形皮疹组成,病程长达20年。从正常或病变/暴露于阳光或未暴露于阳光的皮肤样本中进行了多次皮肤活检。组织病理学检查包括常规苏木精-伊红染色、刚果红染色、硫黄素-T染色和抗细胞角蛋白抗体(AE1、AE3)。结果如下:1)刚果红和硫黄素-T染色阳性表明沉积物具有淀粉样性质;2)淀粉样沉积局限于病变表皮下方(而未累及相邻未受累或远处正常皮肤)表明病变表皮起了一定作用;3)AE3染色阳性进一步表明淀粉样蛋白起源于表皮——II型上皮角蛋白。我们报告一例伴有局部淀粉样沉积的DSP病例,其特征为有阳性家族史、严重瘙痒和肺结核。