Shah Sonal S, Kurago Zoya B
Department of Oral and Maxillofacial Pathology, Radiology and Medicine, New York University College of Dentistry, New York, NY, USA.
N Y State Dent J. 2013 Apr;79(3):46-9.
Many lesions in the oral cavity may present with a papillary or pebbly clinical appearance. Although the great majority of these papillary lesions are histologically diagnosed as squamous or viral papillomas, there are occasional cases of other more unusual possibilities. Angiokeratomas are uncommon vascular lesions that often present clinically as papillomas. They may also present with other varying clinical appearances that range from pigmented lesions to hemangiomas. However, all forms demonstrate a characteristic microscopic appearance consisting of hyperkeratotic, hyperplastic epithelium covering connective tissue with abundant blood vessels that are sharply confined to the connective tissue papillae. Angiokeratomas generally involve the skin and are often associated with an underlying systemic metabolic disease such as Fabry's disease or fucosidosis. Patients with these systemic diseases may have multiple lesions, with possible involvement of the oral mucosa. However, solitary lesions involving only the oral cavity are rare; only eight previous cases have been documented. In this paper, we describe a case of solitary angiokeratoma presenting as a papillary lesion on the ventral tongue of an 18-year-old male. The lesion was surgically excised and no recurrence has been reported to date. Although this patient had no other lesions or systemic issues, we stress the importance of evaluating a patient with a diagnosis of angiokeratoma of the oral cavity for underlying systemic metabolic disease.
口腔中的许多病变可能呈现乳头状或鹅卵石样的临床表现。尽管这些乳头状病变绝大多数在组织学上被诊断为鳞状或病毒性乳头状瘤，但偶尔也有其他更不寻常的可能性。血管角化瘤是罕见的血管性病变，临床上常表现为乳头状瘤。它们也可能呈现出其他不同的临床表现，从色素沉着性病变到血管瘤不等。然而，所有形式都表现出一种特征性的微观表现，即由角化过度、增生的上皮覆盖结缔组织，结缔组织中有丰富的血管，这些血管严格局限于结缔组织乳头。血管角化瘤一般累及皮肤，常与潜在的全身性代谢疾病如法布里病或岩藻糖苷贮积症相关。患有这些全身性疾病的患者可能有多个病变，口腔黏膜也可能受累。然而，仅累及口腔的孤立性病变很少见；此前仅有8例病例报道。在本文中，我们描述了一例18岁男性患者舌腹出现乳头状病变的孤立性血管角化瘤病例。该病变经手术切除，至今未报告复发。尽管该患者没有其他病变或全身性问题，但我们强调对于诊断为口腔血管角化瘤的患者评估其潜在全身性代谢疾病的重要性。
