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一名患有布鲁克-施皮格勒综合征患者头皮上的多个蘑菇样生长的圆柱瘤(头巾瘤):保加利亚患者的独特表现

MULTIPLE MUSHROOM-LIKE GROWING CYLINDROMAS OF THE SCALP (TURBAN TUMOR) IN A PATIENT WITH BROOKE-SPIEGLER SYNDROME: UNIQUE MANIFESTATION IN A BULGARIAN PATIENT.

作者信息

Tchernev G, Broshtilova V, Kordeva S

机构信息

1Onkoderma - Clinic for Dermatology, Venereology and Dermatologic Surgery, Sofia; 2Department of Dermatology and Venereology, Medical Institute of Ministry of Interior, Sofia, Bulgaria.

3Department of Dermatology and Venereology, Medical Military Academy, Sofia, Bulgaria.

出版信息

Georgian Med News. 2024 Oct(355):167-170.

Abstract

Cutaneous cylindromas are rare, slow-growing adnexal tumors commonly found on the capillitium or face. When located on the capillitium, they can cluster together, forming a headgear-like structure that gives the characteristic "turban" appearance. Brooke-Spiegler syndrome, an autosomal dominant condition, is typically benign, though malignant transformation can occur. We present a 61-year-old male with a 30-year history of mushroom-like formations, clinically and histologically confirmed as cylindromas, affecting approximately half of the hairy part of the capillitium. In addition, an erythematous-livid plaque with ulceration and crusting was observed on both left and right lower legs. The patient was suspected of having a sporadic, non-inherited form of Brooke-Spiegler syndrome. Surgical excision of the mushroom-like lesions was recommended. In cases of non-inherited forms of Brooke-Spiegler syndrome, early detection and preventative measures are critical. A brief discussion focusing on the management of the condition is provided, emphasizing whether true sporadic cases of Brooke-Spiegler syndrome exist or if they represent another clinically "silent" form of the condition.

摘要

皮肤圆柱瘤是一种罕见的、生长缓慢的附属器肿瘤,常见于头皮或面部。当位于头皮时,它们可聚集在一起,形成类似头饰的结构,呈现出典型的“头巾”外观。布鲁克 - 施皮格勒综合征是一种常染色体显性遗传病,通常为良性,但可发生恶性转化。我们报告一名61岁男性,有30年蘑菇样肿物病史,经临床和组织学确诊为圆柱瘤,累及头皮毛发部分约一半区域。此外,在双侧小腿观察到有溃疡和结痂的红斑 - 青紫色斑块。该患者被怀疑患有散发性、非遗传性的布鲁克 - 施皮格勒综合征。建议手术切除蘑菇样病变。对于非遗传性的布鲁克 - 施皮格勒综合征病例,早期发现和预防措施至关重要。本文提供了关于该疾病管理的简要讨论,重点在于探讨布鲁克 - 施皮格勒综合征是否存在真正的散发病例,或者它们是否代表该疾病的另一种临床“隐匿”形式。

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