Surgical treatment of facial steatocystoma multiplex

Facial steatocystoma multiplex is a rare variant of steatocystoma multiplex, which is characterized by multiple disfiguring cysts on the face and is inherited in an autosomal dominant fashion. Noninvasive treatments are only temporarily effective, but surgery of multiple lesions on the face has a risk of major complications. A 31-year-old man presented with papules distributed over his face. We used a flap method and mini-incisional capsulectomy under general anesthesia. We performed needle aspiration and CO2 laser puncture and squeezing on the man in the outpatient clinic when symptoms recurred. There have been several reports suggesting various options for the management of steatocystoma. However, long-term management remains difficult and there is no definite technique to treat facial steatocystoma multiplex. We think that combined use of these surgical methods should be applied with individual evaluation. Size, location, hardness, mobility, depth, and previous scars should be noted, and the relevant methods should be selected for each cyst.     

Steatocystoma multiplex: Anatomic reevaluation,electron microscopy,and autoradiography

Summary Histologic and electron-microscopic features of steatocystoma multiplex were investigated. Specimens from 25 patients (8 females, 17 males) were serially cut and three-dimensional drawings constructed. Steatocystoma multiplex is a nevoid sebaceous duct and sebaceous gland tumor, originating from sebaceous follicles, but is not a dermoid tumor. One pilary unit continuously produces vellus hairs, which are trapped in the cystic cavity or in the pilary canal (trichostasis). Steatocystoma multiplex is connected to the epidermis by a straight or meandering epithelial cord, the remnant of the follicular infundibulum. This infundibulum is a more or less solid strand, often containing sebocytes or sebaceous lobulelike structures. A lumen, partly present in a few areas of the cord, is filled with cellular debris of keratinocytes, corneocytes, sebocytes, or trapped hairs.Steatocystoma multiplex has electron-microscopical features similar to the sebaceous duct and sebaceous glands of sebaceous follicles. The ³H-thymidine labeling index of the cyst wall with or without sebaceous acini is lower than in sebaceous follicles. Steatocystoma multiplex suppurativum, characterized by spontaneous rupture of the cyst, inflammation and scarring, mimics acne conglobata with hidradenitis-suppurativa-like lesions as seen in the acne triade or tetrade.Prof. H. H. Wolff is now Chairman of Department of Dermatology, Medizinische Hochschule, D-2400 Lübeck, Federal Republic of Germany     

A Case of Steatocystoma Simplex Involving the Scalp

Steatocystoma is a benign adnexal tumor originating from the pilosebaceous duct junction which can be classified into two groups (steatocystoma simplex and steatocystoma multiplex). Steatocystoma simplex, which presents as a solitary lesion, is very rare. Steatocystoma simplex occurs most commonly on the face and the case reported herein involving the scalp is extremely rare. A 49-year-old man presented for evaluation and treatment of a solitary papule on the right parietal scalp which had persisted for a period of 1 year. The histopathologic examination revealed a thin-walled cyst consisting of stratified squamous epithelium with hyaline cuticle that lacked a stratum granulosum. Based on clinical and histologic findings, we diagnosed this case as steatocystoma simplex of the scalp and report this rare case.     

A Case of Steatocystoma Multiplex Limited to the Scalp

We report here on a 63-year-old woman who had several small, yellowish papules on the scalp for the previous 2 years. There was no family history of similar lesions. Yellowish, creamy material was expressed from a papule during punch biopsy. Histologic examination from the lesion revealed the typical features of steatocystoma multiplex. We report here on this rare variant of steatocystoma multiplex that was limited to the scalp.     

Mammographic demonstration of steatocystoma multiplex

Steatocystoma multiplex (SM) is a rare inherited cutaneous disorder characterised by multiple subcutaneous oil cysts. We present the radiological features of this uncommon condition in a case report. Numerous lucent, well-circumscribed, smooth-walled nodules appeared in both breasts at mammography. Many lipid-containing intradermal cysts of different sizes were also observed at sonography, scattered over both breasts, axillae, sternal region and abdomen. SM was suspected and no further evaluations were made. Received 9 June 1997; Revision received 6 August 1997; Accepted 7 August 1997     

多发性脂囊瘤角蛋白17基因的突变研究

目的:研究多发性脂囊瘤(SM)角蛋白17基因的突变,为进一步开展基因诊断和基因治疗奠定基础。方法:抽取多发性脂囊瘤患者、患者父母以及100例正常人静脉血提取基因组DNA,采用聚合酶链反应(PCR)的方法对角蛋白17外显子1及其侧翼和外显子6进行扩增,并对其PCR产物直接进行双向测序以检测突变。结果:在家系中两例患者第42位密码子由CTG突变为CCG,结果导致角蛋白17 V1区杂合错义突变(L42P),即亮氨酸由脯氨酸替代,而此家系中的正常人和与该家系无关的100例正常人的DNA测序结果未发现此突变。结论:此家系中患者表型可能由角蛋白17基因头区的L42P突变所致。     

Fractionated ablative carbon dioxide laser treatment of steatocystoma multiplex

Steatocystoma multiplex is a well-recognized condition in which subjects develop dermal cysts generally inherited in an autosomal dominant fashion, though these can occur sporadically. This case report describes the successful treatment of a 51-year-old woman with steatocystomata limited to the face, who after two treatments with a fractionated ablative carbon dioxide laser remained free of cysts for three years. We conclude that this treatment should be considered as an efficient and effective treatment option for patients with steatocystoma multiplex.     

Steatocystoma multiplex as initial impression of non-small cell lung cancer with complete response to gefitinib

Cutaneous metastases are rare and seldom present at the time of first diagnosis of cancer. Data from various studies show that 1-12% of lung cancer patients experience tumor spread to the skin. The scalp, chest, and abdomen are favored sites of skin metastases from lung cancers, but metastases to multiple skin sites in a single patient are rarely reported. We describe a 56-year-old lung adenocarcinoma patient, initially diagnosed with steatocystoma multiplex who responded well to gefitinib treatment. The efficacy of conventional chemotherapy for cutaneous metastases has been limited because of the relatively poor blood supply to the skin. It has been demonstrated that tyrosine kinase inhibitor (TKI), gefitinib, has significant clinical benefit in lung cancer patients with epidermal growth factor receptor (EGFR) mutation even in metastases to the brain. However, the therapeutic response to gefitinib in patients with skin metastases is seldom mentioned in the literature. We report one case of lung adenocarcinoma with multiple skin metastases that were successfully treated with gefitinib.     

脂囊瘤的超声特征分析

目的分析脂囊瘤临床及超声特征。方法回顾性分析57例经手术病理证实的脂囊瘤的临床及超声特征。结果 57例脂囊瘤患者,47例为多发(82.46%)(≥2个团块),10例为单发(17.54%),二维超声:超声检查并经手术切除共58个团块,均表现为皮肤及皮下层无回声团,其中位于头面部25个(43.10%),四肢18个(31.03%),躯干及颈部15个(25.87%),团块最大直径5~42mm,平均(13.61±7.73)mm,边界清楚54个(93.10%),形态规则54个(93.10%),单纯无回声团1个(1.73%),无回声团伴高回声57个(98.27%),后方回声增强56个(96.54%),所有团块内部均未见钙化,CDFI示0级56个(96.54%),Ⅰ级和Ⅱ级均为1个(1.73%),Ⅲ级0个。结论脂囊瘤好发于青年头面部及四肢,常多发,临床表现为无痛性体表包块,超声表现具有一定特征性,可作为本病首选影像学检查方法,掌握其临床及超声特征有助于提高诊断准确率。