Cardona Rocio, Cancel-Artau Karina J, Carrasquillo Osward Y, Martin-Garcia Rafael F
Department of Dermatology, University of Puerto Rico School of Medicine, San Juan, PR ; and.
University of Puerto Rico School of Medicine, San Juan, PR.
Am J Dermatopathol. 2021 Jan 1;43(1):67-70. doi: 10.1097/DAD.0000000000001720.
Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by cutaneous and extracutaneous hamartomas. Dermatologic evaluation is critical for early diagnosis because mucocutaneous manifestations account for 4 of 11 major and 3 of 6 minor diagnostic criteria. Folliculocystic and collagen hamartoma (FCCH) is a recently described entity associated with TSC. We herein describe the case of a 28-year-old woman with a history of TSC who presented with a scalp lesion present since childhood. Physical examination revealed a solitary, well-circumscribed exophytic tumor over the occipital scalp measuring 9 × 8 cm and covered with comedones and cyst-like structures. Biopsy of the lesion demonstrated thickening of the collagen bundles throughout the dermis, concentric perifollicular and perivascular fibrosis, an increased number of dilated vessels, and keratin-filled cysts lined by the infundibular epithelium. Clinicopathologic correlation was diagnostic for FCCH. The patient was referred for surgical excision. In addition, we review 11 other cases of FCCH previously reported in the literature.
结节性硬化症（TSC）是一种以皮肤和皮肤外错构瘤为特征的神经皮肤疾病。皮肤评估对于早期诊断至关重要，因为黏膜皮肤表现占11项主要诊断标准中的4项以及6项次要诊断标准中的3项。毛囊囊肿性和胶原性错构瘤（FCCH）是一种最近描述的与TSC相关的病变。我们在此报告一例28岁有TSC病史的女性，其自童年起就有头皮病变。体格检查发现枕部头皮有一个孤立的、边界清楚的外生性肿瘤，大小为9×8 cm，表面有黑头粉刺和囊肿样结构。病变活检显示整个真皮层胶原束增厚，毛囊周围和血管周围同心性纤维化，扩张血管数量增加，以及由漏斗状上皮衬里的充满角蛋白的囊肿。临床病理相关性诊断为FCCH。该患者被转诊接受手术切除。此外，我们还回顾了文献中先前报道的其他11例FCCH病例。
