Saggini Andrea, Mully Thaddeus
Department of Dermatology, University of Rome Tor Vergata, Rome, Italy.
J Cutan Pathol. 2014 Apr;41(4):380-5. doi: 10.1111/cup.12276. Epub 2014 Jan 30.
We report the unprecedented case of reactive eccrine syringofibroadenoma (ESFA) secondary to primary cutaneous amyloidosis. A 62-year-old woman of Asian ethnicity presented with a pruritic rash on the back of long-standing duration. Physical examination revealed diffuse hyperpigmentation localized to the interscapular region; there were a multitude of hyperpigmented macules merged in a rippled pattern intermixed with scattered papules and cobblestone-like areas. A punch biopsy from a papule was taken. Histopathological examination revealed a network of epithelial strands and cords hanging from the epidermis and harboring foci of ductal differentiation. Eosinophilic collections of amorphous material were found between the epithelial strands, obscuring the superficial dermis. The microscopic picture was consistent with primary cutaneous amyloidosis associated with reactive ESFA. Results of histochemical and immunohistochemical staining confirmed the diagnosis. We speculate that pathogenetic mechanisms intrinsic to primary cutaneous amyloidosis, in addition to unknown genetic factors, resulted in clinical changes of lichen amyloidosus associated with an abnormal hyperplastic epithelial response with histopathological features of ESFA rather than the common epidermal change of acanthosis and hyperkeratosis.
我们报告了一例继发于原发性皮肤淀粉样变的反应性小汗腺汗管纤维腺瘤（ESFA）的罕见病例。一名62岁的亚洲女性，背部出现持续时间较长的瘙痒性皮疹。体格检查发现肩胛间区弥漫性色素沉着；有大量色素沉着斑呈波纹状融合，夹杂着散在的丘疹和鹅卵石样区域。从一个丘疹处取了打孔活检组织。组织病理学检查显示，上皮条索和索状结构从表皮垂下，有导管分化灶。在上皮条索之间发现嗜酸性无定形物质聚集，使浅表真皮模糊不清。显微镜下表现符合与反应性ESFA相关的原发性皮肤淀粉样变。组织化学和免疫组织化学染色结果证实了诊断。我们推测，除了未知的遗传因素外，原发性皮肤淀粉样变的内在发病机制导致了苔藓样淀粉样变的临床变化，伴有具有ESFA组织病理学特征的异常增生性上皮反应，而非常见的棘层增厚和角化过度的表皮变化。
