Nam Chan Hee, Park Min Kee, Choi Mi Soo, Hong Seung Phil, Park Byung Cheol, Kim Myung Hwa
Department of Dermatology, Dankook University Medical College, Cheonan, Korea.
Ann Dermatol. 2017 Feb;29(1):79-82. doi: 10.5021/ad.2017.29.1.79. Epub 2017 Feb 3.
Secondary cutaneous amyloidosis refers to clinically unapparent amyloid deposits within the skin in association with a pre-existing skin condition or skin tumors, such as basal cell carcinoma, porokeratosis, solar elastosis, Bowen's disease, and mycosis fungoides. A 70-year-old woman presented with a 6-month history of asymptomatic multiple yellowish plaques on both legs. She had been diagnosed with mycosis fungoides 7 years ago and was treated with psoralen and ultraviolet A radiation (PUVA) therapy, narrow-band ultraviolet B (UVB) therapy, and acitretin for 5 years. Finally, she reached complete remission of mycosis fungoides. However, new yellowish lesions started to appear 1 year after discontinuing the phototherapy. A physical examination revealed multiple yellowish plaques on both extremities. The plaques were well circumscribed and slightly elevated. All laboratory tests were normal. A biopsy specimen showed multiple nodular deposits of eosinophilic amorphous material in papillary dermis and upper reticular dermis. The deposits represented apple green birefringence on Congo red stain viewed under polarized light. Acellular small nodules in the upper dermis consisted of randomly oriented, non-branching, 6.67~12.7 nm thick amyloid fibrils on electron microscopy. We report an interesting and rare case of secondary cutaneous amyloidosis after narrow-band UVB therapy and PUVA therapy in a patient with mycosis fungoides.
继发性皮肤淀粉样变是指在皮肤内临床上不明显的淀粉样沉积物,与先前存在的皮肤疾病或皮肤肿瘤相关,如基底细胞癌、汗孔角化症、日光性弹力组织变性、鲍温病和蕈样肉芽肿。一名70岁女性双下肢出现无症状的多发淡黄色斑块6个月。她7年前被诊断为蕈样肉芽肿,接受补骨脂素和紫外线A辐射(PUVA)疗法、窄谱中波紫外线(UVB)疗法和阿维A治疗5年。最终,她的蕈样肉芽肿达到完全缓解。然而,停止光疗1年后开始出现新的淡黄色皮损。体格检查发现双下肢有多发淡黄色斑块。斑块边界清晰,略隆起。所有实验室检查均正常。活检标本显示乳头真皮层和上部网状真皮层有多个嗜酸性无定形物质的结节状沉积物。在偏振光下观察,刚果红染色显示沉积物呈苹果绿双折射。电镜检查显示真皮上部的无细胞小结节由随机排列、无分支、6.67~12.7nm厚的淀粉样原纤维组成。我们报告了1例蕈样肉芽肿患者在窄谱UVB疗法和PUVA疗法后发生继发性皮肤淀粉样变的罕见有趣病例。