J Korean Assoc Oral Maxillofac Surg.  2014 Oct;40(5):240-245. 10.5125/jkaoms.2014.40.5.240.

Angiokeratoma circumscriptum of the buccal mucosa: a case report and literature review

• Kang YH
• Byun JH
• Park BW

• KMID: 2005454
• DOI: http://doi.org/10.5125/jkaoms.2014.40.5.240

Abstract

Angiokeratoma is a benign cutaneous lesion of the capillaries, presenting as dilated vessels in the upper part of the dermis. Although this disorder is classified into various types and has been occasionally reported in the skin of the scrotum or extremities, the involvement of the oral cavity mucosa has been rarely reported. The present study reports a case of angiokeratoma circumscriptum in the buccal mucosa. The expression of vascular endothelial growth factor (VEGF) and both of its receptors (VEGFR-1 and VEGFR-2) was demonstrated by immunohistochemistry in the endothelial cells lining the dilated vessels. The expression of VEGFR-2 was higher than that of VEGFR-1 in the endothelial cells in the lesion, indicating an increased rate of endothelial cell proliferation within the lesion. Interestingly, some of the endothelial cells co-expressed VEGF and its two receptors. These results suggest that endothelial cells in the pathologically dilated vessels possess VEGF autocrine growth activity involved in vasculogenesis and maintenance in angiokeratoma lesions. To our knowledge, this is the second report published on isolated oral angiokeratoma confined to the buccal mucosa and the first case report on angiokeratoma circumscriptum involving the buccal mucosa.

Keyword

Angiokeratoma circumscriptum; Buccal mucosa

MeSH Terms

Angiokeratoma*
Capillaries
Dermis
Endothelial Cells
Extremities
Immunohistochemistry
Mouth
Mouth Mucosa*
Mucous Membrane
Scrotum
Skin
Vascular Endothelial Growth Factor A
Vascular Endothelial Growth Factor Receptor-1
Vascular Endothelial Growth Factor Receptor-2
Vascular Endothelial Growth Factor A
Vascular Endothelial Growth Factor Receptor-1
Vascular Endothelial Growth Factor Receptor-2

Figure

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  Fig. 1 Preoperative clinical and magnetic resonance imaging (MRI) findings. A. Primary lesions presented as multiple, small, irregular, raised lesions on the right side of the buccal mucosa. B, C. MRI shows an ill-defined heterogeneously enhanced lesion on the right side of the cheek (arrows).

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  Fig. 2 H&E staining showing the histopathological features of the angiokeratoma circumscriptum lesion on the buccal mucosa (scale bars=200 µm). A. Low-power magnification shows epithelial hyperkeratosis, acanthosis, and large cystic lesions. B. High-power magnification shows erythrocytes filling the dilated cystic lesion. These pathological features are consistent with those of angiokeratoma circumscriptum.

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  Fig. 3 Intraoperative and postoperative views. A. The cheek lesion was excised, and the surgical defect was primarily closed with an advancement flap. B. Two months after the operation, the surgical site was healed uneventfully.

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  Fig. 4 Immunohistochemistry for vascular endothelial growth factor (VEGF) and both of its receptors (VEGFR-1 and VEGFR-2) (scale bars=100 µm). VEGF, VEGFR-1, and VEGFR-2 were expressed in endothelial cells and stromal cells in the lesion. Staining was stronger and the proportion of positive cells was higher for VEGFR-2 than for VEGFR-1. Several lesion cells co-expressed VEGF as well as VEGFR-1 and VEGFR-2 (arrows). In addition, some additional endothelial cells stained positive for VEGF and VEGFR-2, but not for VEGFR-1 (arrowheads). The coexpression of VEGF and its receptors suggests that VEGF is an autocrine growth factor for endothelial cells in the oral angiokeratoma circumscriptum lesion presented in this study.

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