Lowitt M H, Kariniemi A L, Niemi K M, Kao G F
Department of Dermatology, University of Maryland School of Medicine, Baltimore 21201, USA.
J Am Acad Dermatol. 1996 Feb;34(2 Pt 2):325-32. doi: 10.1016/s0190-9622(07)80002-8.
Malacoplakia, an inflammatory disease characterized by accumulations of phagocytic macrophages, occurs primarily in immunocompromised individuals. Cutaneous involvement is rare. Two men, each with a renal allograft, had expanding nodules on the temple and perianal area (case 1) and perianal, inguinal, and scrotal skin (case 2). Lesions resolved after combined surgical and antibiotic therapy. Histopathologic examination showed dense infiltration with large phagocytic macrophages containing round, concentric, laminar Von Kossa stain-positive inclusion bodies. Histiocytes had positive results for CD 68, lysozyme, and alpha 1-antitrypsin. Electron microscopic examination demonstrated rare intracytoplasmic inclusion bodies with concentric electron-dense laminations of calcium (Michaelis-Gutmann bodies.) Cutaneous malacoplakia should be considered in the differential diagnosis of nodules or draining ulcers, particularly in immunocompromised patients. Because Michaelis-Gutmann bodies are difficult to identify, specimens should be evaluated for cutaneous malacoplakia by immunohistochemical or electron microscopic means.
软斑病是一种以吞噬性巨噬细胞聚集为特征的炎症性疾病,主要发生于免疫功能低下者。皮肤受累罕见。两名接受肾移植的男性,分别在颞部和肛周区域(病例1)以及肛周、腹股沟和阴囊皮肤(病例2)出现逐渐增大的结节。经手术和抗生素联合治疗后病变消退。组织病理学检查显示大量吞噬性巨噬细胞密集浸润,这些巨噬细胞含有圆形、同心、层状的Von Kossa染色阳性包涵体。组织细胞的CD 68、溶菌酶和α1 -抗胰蛋白酶检测结果为阳性。电子显微镜检查显示罕见的胞质内包涵体,有同心的电子致密钙层(Michaelis - Gutmann小体)。在结节或引流性溃疡的鉴别诊断中,尤其在免疫功能低下患者中,应考虑皮肤软斑病。由于Michaelis - Gutmann小体难以识别,应通过免疫组织化学或电子显微镜方法对标本进行皮肤软斑病评估。