Ramos-Ceballos Frances I, Pashaei Shayesteh, Kincannon Jay M, Morgan Michael B, Smoller Bruce R
Department of Dermatology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA.
J Cutan Pathol. 2008 May;35(5):477-83. doi: 10.1111/j.1600-0560.2007.00832.x.
Generalized basaloid follicular hamartoma syndrome (GBFHS) is a rare, recently-described, autosomal-dominantly inherited disorder that presents with disseminated milia, palmoplantar pitting, hypotrichosis and basaloid follicular hamartomas (BFH). BFH is a benign adnexal tumor that resembles basal cell carcinoma (BCC). In this study, we report two cases of GBFHS and stain BFH, a vellus hair hamartoma (VHH) and a neurofollicular hamartoma (NH) with CD34, bcl-2 and CD10 to characterize and compare the staining patterns of these follicular tumors. Standard immunohistochemistry labeling with CD34, bcl-2 and CD10 was performed on paraffin-embedded, formalin-fixed tissue sections of five BFH (four for CD10), one VHH and one NH. CD34 stromal staining was observed in all specimens. Bcl-2 stained the outermost cell layers of the basaloid nests in all specimens. CD10 stained the peritumoral stroma of all specimens. The BFH, NFH and the VHH showed CD10 staining of matrical cells. CD34 and CD10 stain peritumoral stroma of BFH, VHH and NH. Bcl-2 stains the outermost cell layer of these tumors. CD10 was also observed to stain matrical cells. These results show the similarities in differentiation between these benign follicular neoplasms and trichoepithelioma.
泛发性基底样毛囊错构瘤综合征(GBFHS)是一种罕见的、最近才被描述的常染色体显性遗传性疾病,其表现为播散性粟丘疹、掌跖点状凹陷、毛发稀少和基底样毛囊错构瘤(BFH)。BFH是一种类似于基底细胞癌(BCC)的良性附属器肿瘤。在本研究中,我们报告了2例GBFHS病例,并对BFH、毳毛毛囊错构瘤(VHH)和神经毛囊错构瘤(NH)进行CD34、bcl-2和CD10染色,以表征和比较这些毛囊肿瘤的染色模式。对5例BFH(4例用于CD10染色)、1例VHH和1例NH的石蜡包埋、福尔马林固定组织切片进行CD34、bcl-2和CD10的标准免疫组织化学标记。在所有标本中均观察到CD34间质染色。在所有标本中,bcl-2对基底样巢的最外层细胞层进行染色。CD10对所有标本的肿瘤周围间质进行染色。BFH、NFH和VHH的基质细胞显示CD10染色。CD34和CD10对BFH、VHH和NH的肿瘤周围间质进行染色。bcl-2对这些肿瘤的最外层细胞层进行染色。还观察到CD10对基质细胞进行染色。这些结果显示了这些良性毛囊肿瘤与毛发上皮瘤在分化方面的相似性。