Enbiale Wendemagegn, Tirumalae Rajalakshmi, Böer-Auer Almut
Federal Ministry of Health Ethiopia, Addis Abeba, Ethiopia.
St. John's Medical College, Bangalore, India.
J Cutan Pathol. 2015 Mar;42(3):173-181. doi: 10.1111/cup.12441. Epub 2015 Feb 5.
Podoconiosis is a familial geochemical dermatosis which is common in Ethiopia but relatively unknown in Europe/United States. It is related to exposure of bare feet to volcanic soil and presents with extensive bilateral lymphedema of legs and feet. Histopathological and immunohistochemical features of it have not been described yet.
The objectives of this study are to characterize podoconiosis histopathologically and immunohistochemically and to increase awareness of the disease.
Ten specimens of fully developed podoconiosis were examined with hematoxylin/eosin, periodic acid-Schiff, Gram, elastica-van Gieson stainings, with immunohistochemistry (CD3,CD20,CD31,CD68,CD138, tryptase, podoplanin, collagen IV), and with polymerase chain reaction (PCR) for human papillomavirus (HPV)-specific DNA.
All specimens showed verrucous acanthosis and papillomatosis. Eccrine ducts demonstrated hyperplasia, syringofibroadenomatous changes and miliaria. Dermal collagen bundles were thickened, and elastic fibers were dramatically reduced. A moderate lymphoplasmacytic infiltrate was joined by mast cells and scattered macrophages; neutrophils and eosinophils were sparse. Blood vessels were increased, dilated, and often sclerotic while lymphatics were reduced and largely not dilated. HPV-PCR was negative in all specimens.
Podoconiosis demonstrates distinctive changes of chronic lymphedema with extensive sclerosis, loss of elastic fibers, verrucous acanthosis (not HPV induced) and reactive changes of eccrine structures. Mast cells, macrophages and altered blood vessels may be involved in the pathogenesis.
足分支菌病是一种家族性地球化学性皮肤病,在埃塞俄比亚很常见,但在欧洲/美国相对鲜为人知。它与赤足接触火山土壤有关,表现为双下肢和足部广泛的淋巴水肿。其组织病理学和免疫组化特征尚未见报道。
本研究的目的是对足分支菌病进行组织病理学和免疫组化特征描述,并提高对该疾病的认识。
对10例完全发展的足分支菌病标本进行苏木精/伊红染色、过碘酸-希夫染色、革兰染色、弹性纤维-范吉森染色、免疫组化(CD3、CD20、CD31、CD68、CD138、类胰蛋白酶、足板蛋白、IV型胶原)以及人乳头瘤病毒(HPV)特异性DNA的聚合酶链反应(PCR)检测。
所有标本均显示疣状棘皮症和乳头瘤病。小汗腺导管增生、呈汗腺纤维腺瘤样改变及粟丘疹形成。真皮胶原束增厚,弹性纤维显著减少。有中度淋巴细胞和浆细胞浸润,并伴有肥大细胞和散在的巨噬细胞;中性粒细胞和嗜酸性粒细胞稀少。血管增多、扩张且常呈硬化状态,而淋巴管减少且大多不扩张。所有标本的HPV-PCR检测均为阴性。
足分支菌病表现为慢性淋巴水肿的独特变化,伴有广泛硬化、弹性纤维丧失、疣状棘皮症(非HPV诱导)和小汗腺结构的反应性改变。肥大细胞巨噬细胞和血管改变可能参与了其发病机制。