Hazemann G, Michel C, Mahé A, Lipsker D, Cribier B
Clinique dermatologique, université de Strasbourg, hôpitaux universitaires de Strasbourg, 1, place de l'Hôpital, 67091 Strasbourg cedex, France.
Centre hospitalier Emile-Muller, 20, avenue du Dr-René-Laennec, 68100 Mulhouse, France.
Ann Dermatol Venereol. 2019 Mar;146(3):181-191. doi: 10.1016/j.annder.2018.12.007. Epub 2019 Jan 26.
Basaloid follicular hamartoma (BFH) is a rare tumor first described in 1985. It bears clinical and histologic similarities with basal cell carcinoma (BCC), in particular the so-called infundibulocystic form. We performed a single-center clinicopathological study of a series of typical cases of this entity that is occasionally difficult to diagnose.
All cases of BFH seen at the Dermatopathology Laboratory of Strasbourg University Hospital were included and analyzed by means of HE staining and Ber-EP4 and PHLDA1 immunolabelling. Diagnosis was made in the event of basaloid proliferation with anastomosing cords developed from a hair follicle. Clinical data were collected from clinical files.
We identified 15 cases in 13 patients of mean age 44.8 years (range: 4 to 90) and the sex-ratio was 5/8. Lesions consisted of flesh-colored papules measuring 0.3 to 1.2cm in diameter, without any preferential site. Three patients had multiple lesions consisting of several coalescent papules on a breast for one, sparse papules on the back for another, and hundreds of linear unilateral BFHs, associated with osseous abnormalities, characteristic of Happle-Tinschert syndrome, for the third. All tumors were limited to the superficial and mid dermis, with a vertical orientation and connection to the epidermis in 14 of the 15 patients. In some cases, the outermost cells were basophilic while the central cells were eosinophilic. Peripheral palisading and clefting were frequently observed. Keratin cysts or sebaceous ducts were consistently present, indicating follicular differentiation. Ber-EP4+ cells were restricted to the periphery of the cords and PHLDA1 was weakly expressed.
BFH is a rare entity that must be differentiated from BCC. It presents as solitary or multiple lesions, either grouped in plaques or with a generalized or linear unilateral distribution. Generalized BFH may be associated with autoimmune diseases and linear unilateral BFH with osseous, dental and cerebral abnormalities in Happle-Tinschert syndrome. It is important to distinguish BFH from BCC to avoid inappropriate aggressive treatment.
基底样毛囊错构瘤（BFH）是一种罕见肿瘤，于1985年首次被描述。它在临床和组织学上与基底细胞癌（BCC）相似，尤其是所谓的漏斗状囊肿型。我们对这一有时难以诊断的实体的一系列典型病例进行了单中心临床病理研究。
纳入斯特拉斯堡大学医院皮肤病理学实验室所见的所有BFH病例，并通过苏木精-伊红（HE）染色以及Ber-EP4和PHLDA1免疫标记进行分析。若出现由毛囊形成的相互吻合索状的基底样增生，则做出诊断。临床数据从临床病历中收集。
我们在13例患者中识别出15个病例，平均年龄44.8岁（范围：4至90岁），男女比例为5/8。皮损由直径0.3至1.2厘米的肉色丘疹组成，无任何好发部位。3例患者有多发皮损，1例患者乳房上有几个融合的丘疹，另1例患者背部有散在丘疹，第3例患者有数百个单侧线状BFH，伴有骨异常，为哈普尔-廷舍特综合征的特征表现。所有肿瘤均局限于真皮浅层和中层，15例患者中有14例呈垂直方向并与表皮相连。在某些病例中，最外层细胞为嗜碱性，而中央细胞为嗜酸性。经常观察到外周栅栏状排列和裂隙形成。始终存在角质囊肿或皮脂腺导管，表明有毛囊分化。Ber-EP4阳性细胞局限于索状结构的周边，PHLDA1呈弱表达。
BFH是一种罕见实体，必须与BCC相鉴别。它表现为单发或多发皮损，可呈斑块状聚集或呈泛发性或单侧线状分布。泛发性BFH可能与自身免疫性疾病相关，单侧线状BFH与哈普尔-廷舍特综合征中的骨、牙齿和脑部异常相关。将BFH与BCC区分开来很重要，以避免不适当的积极治疗。
