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脂肪性水肿性脱发:一种罕见的非瘢痕性但永久性脱发的临床病理变异型。

Lipedematous alopecia: an uncommon clinicopathologic variant of nonscarring but permanent alopecia.

作者信息

González-Guerra Elena, Haro Rosario, Angulo Jorge, Del Carmen Fariña Maria, Martín Lucia, Requena Luis

机构信息

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.

出版信息

Int J Dermatol. 2008 Jun;47(6):605-9. doi: 10.1111/j.1365-4632.2008.03405.x.

Abstract

A 52-year-old black woman presented with a 5-year history of gradual swelling and slowed hair growth involving the vertex and both parietal regions of the scalp. Gradually, the swelling progressed to involve the entire scalp, only sparing a peripheral crown. She reported no history of trauma or medications. Slight pruritus of the involved area was the only accompanying symptom. There was no family history of a similar condition. Her past medical history included surgery for ovarian cysts, 10 years previously, and cholelithiasis. Physical examination revealed diffuse hair thinning and alopecia, more prominent along the vertex and parietal regions (Fig. 1a). There was no evidence of scalp inflammation, scaling, or increased hair fragility. The scalp was mildly tender on palpation and had a boggy, spongy consistency. The hairs which still remained in the involved areas were thin, short, and soft (Fig. 1b). The involved area was slightly hypopigmented when compared with adjacent noninvolved scalp. The rest of the physical examination was within normal limits. No abnormalities of the hair shaft were observed on microscopic examination of several plucked hairs. Laboratory investigations, including a complete blood cell count, blood chemistry, urinalysis, sedimentation rate, antinuclear antibodies, and serologic tests for syphilis, hepatitis B and C virus, and human immunodeficiency virus, were negative. A computed tomography scan of the skull demonstrated diffuse and regular thickening of subcutaneous fatty tissue of the scalp, disclosing a maximum scalp thickness of 15 mm at the vertex (Fig. 2). The biopsy from the vertex revealed a normal epidermis and dermis, with diffuse loss of hair follicles. The most striking feature consisted of a large increase in thickness of the subcutaneous fatty tissue (Fig. 3a). Pre-existing hair follicles were replaced by vertical fibrous tracts of lamellar fibroplasia with no inflammatory infiltrate (Fig. 3b). Adipocytes showed a normal size and shape, but the connective tissue septa, which are normally present separating the subcutaneous tissue into fat lobules, were lacking, and subcutaneous fatty tissue consisted of a continuous and diffuse sheet of mature adipocytes (Fig. 3c). Orcein stain revealed normal contents of elastic fibers with foci of condensation at the sites of disappeared pre-existing hair follicles (Fig. 3d). Colloidal iron and Alcian blue (pH 2.5) stains revealed no mucin deposits.

摘要

一名52岁的黑人女性,头皮顶部及双侧顶叶区域逐渐肿胀且头发生长缓慢,已有5年病史。肿胀逐渐蔓延至整个头皮,仅外周冠状区域未累及。她否认有外伤史及用药史。受累区域仅有轻微瘙痒这一伴随症状。家族中无类似疾病史。她既往有卵巢囊肿手术史(10年前)及胆石症病史。体格检查发现弥漫性头发稀疏及脱发,在顶部和顶叶区域更为明显(图1a)。无头皮炎症、脱屑或头发脆性增加的证据。触诊时头皮轻度压痛,质地呈软泥状、海绵样。受累区域残留的头发细、短且软(图1b)。与相邻未受累头皮相比,受累区域略显色素减退。其余体格检查结果正常。对多根拔除毛发进行显微镜检查,未观察到毛干异常。实验室检查,包括全血细胞计数、血液生化、尿液分析、血沉、抗核抗体以及梅毒、乙型和丙型肝炎病毒、人类免疫缺陷病毒的血清学检测,结果均为阴性。头颅计算机断层扫描显示头皮皮下脂肪组织弥漫性、对称性增厚,顶部头皮最大厚度达15毫米(图2)。顶部活检显示表皮和真皮正常,但毛囊弥漫性缺失。最显著的特征是皮下脂肪组织厚度大幅增加(图3a)。原有的毛囊被垂直的板层状纤维增生纤维束取代,无炎症浸润(图3b)。脂肪细胞大小和形态正常,但正常情况下将皮下组织分隔成脂肪小叶的结缔组织间隔缺失,皮下脂肪组织由连续、弥漫的成熟脂肪细胞片组成(图3c)。地衣红染色显示弹性纤维含量正常,在原有毛囊消失部位有凝聚灶(图3d)。胶态铁和阿尔辛蓝(pH 2.5)染色未发现黏蛋白沉积。

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