Volume 4, Issue 4 , Pages 173-176, December 2009
Tufted angioma in the upper lip: A report of a case
Article Outline
Summary
Tufted angioma is a rare benign vascular tumor of endothelial origin that is seldom seen in the oral mucosa. We described a case of a tufted angioma observed in a 12-year-old male from an indigenous Guarani community that was clinically diagnosed as an oral manifestation of primary syphilis. We discussed lesions to be considered as reasonable diagnostic possibilities and how the diagnosis was achieved. We concluded that in spite of being less frequent in the oral mucosa, tufted angioma must be considered in the differential diagnosis of oral proliferative lesions.
Keywords: Tufted angioma, Vascular tumor, Vermillion of the lip
1. Introduction
Tufted angioma is a rare benign vascular tumor of endothelial origin that generally manifests during the first year of life and which can, in some cases, be congenital [1], [2], [3], and which occasionally appears among adults [4], [5], [6]. Clinically, there is no gender-based predisposition and the lesions present in a variety of forms, as hard papules or nodules, of red, brown or violet color, primarily located on the skin of the neck, shoulders and thorax, of varying size, from small lesions [4], to lesions of more than 5
cm in diameter [1]. They grow slowly and cause painful sensitivity. Hyperhidrosis of the adjacent skin and hypertrichosis may be present [1], [2]. There are reports of spontaneous remission in congenital cases [3].
The medical literature contains four case reports of TA located in the oral mucosa [2], [4], [5]. Of these four cases in the mouth, two were located in the mucosa of the upper lip, close to the vermillion [4], [5], one on the floor of the mouth [5], and one on the jugal mucosa, extending from the area of the commissure to the mandibular angle [2].
2. Case report
A 12-year-old male from the indigenous Guarani community in Saltinho, RS, Brazil, had been presented a swelling in the upper lip for 2 months. The patient reported an episode of bleeding 20 days prior to this, but clinical history was complicated by difficulties in communicating with him since he spoke very little Portuguese and there were also cultural barriers to communication. Clinical examination revealed a nodular lesion, located on the vermillion of the upper right lip, close to the medial line with an ulcerated surface and hardened edges, predominantly brown in color with reddened and yellowed areas, with an 8mm diameter, asymptomatic and with bilateral submandibular lymphadenopathy (Fig. 1). The patient's medical history was noncontributory. The lesion was diagnosed clinically as syphilitic chancre or Pyogenic Granuloma.
Fig. 1.
Clinical examination revealed a nodular lesion, located on the vermillion of the upper right lip, close to the medial line with an ulcerated surface and hardened edges, predominantly brown in color with reddened and yellowed areas. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
During the diagnostic process, laboratory tests were requested. Hematological parameters investigated included erythrocytes, hematocrit, hemoglobin, leukocytes, and platelets, all of which were within normal limits. The patient was seronegative for HIV-I and II and neither VDRL or FTA-ABS tests reacted.
When the patient attended his second appointment the lesion had reduced in volume (6mm in diameter) and its surface had been covered by normal mucosa. Another diagnostic consideration included Cutaneous Leishmaniasis, which presents a degree of clinical diagnostic difficulty that makes biopsy and histopathological examination mandatory [6].
An incisional biopsy was therefore taken of the lesion and the resulting specimens revealed irregular nodules or tufts of capillary sized vessels in a “cannon ball” fashion on microscopic analysis, as described by Enzinger and Weiss in 2001 [7] (Fig. 2). Some of the capillaries had become condensed, resulting in solid areas. Vascular spaces had formed within these clusters, with a single cell wall and a slit-shaped lumen. The vascular spaces can vary from poorly canalized capillaries to large vessels with thin walls and an endothelial covering of a single layer of cells. The endothelial cells exhibited dark nuclei and had grown into the interior of the vessels. Peripheral cells had nuclei of greater volume that were palely stained by the hematoxylin and with a fusiform or polygonal pattern, reminiscent of pericytes (Fig. 3). Some of the capillaries had become condensed, resulting in solid areas of endothelial cells and pericytes. Fig. 4 shows that the process is deep within the reticular dermis and subcutaneous tissue and in some areas grows in an irregular fashion throughout the dermis. Occasional polymorphonuclear leukocytes nuclei were also observed. Neither atypical cells nor mitotic activity were present. The diagnosis was tufted angioma.
Fig. 2.
Microscopic analysis showing irregular nodules or tufts of capillary sized vessels in a “cannon ball” fashion deep within the reticular dermis and subcutaneous tissue (white arrows) (hematoxylin-eosin; original magnification 40×).
Fig. 3.
The black arrow shows endothelial cells exhibited dark nuclei and had grown into the interior of the vessels, and the white arrow shows peripheral cells had nuclei of greater volume palely stained with a polygonal pattern, reminiscent of pericytes (hematoxylin-eosin; original magnification 400×).
Fig. 4.
The white arrows show solid areas growing in an irregular fashion throughout the dermis (hematoxylin-eosin; original magnification 40×).
The patient abandoned treatment and could not be located, being therefore lost to follow-up.
3. Discussion
The majority of TA of the mouth described in literature were small, painless and covered by mucosa. The lesion described here presented as a deep submucosal nodule, resulting in a firm, painless elevation, with an ulcerated surface, on the vermillion of the upper lip. The clinical presentation, size, location and symptomatology of the lesion initially suggested a clinical diagnosis of syphilitic chancre that is a manifestation of primary syphilis. The diagnosis of primary syphilis is made by clinical examination in combination with laboratory tests. The laboratory tests were important, not only to assess the chances of a diagnosis of primary syphilis, but also to rule out Kasabach–Merritt syndrome, that is a rare consumptive coagulopathy of an aggressive nature, which typically occurs during childhood and which has been associated with TA. This coagulopathy results in thrombocytopenia, anemia and hypofibrinogenemia [8].
The bleeding history together with the ulcerated surface of the lesion could have indicated a diagnosis of Pyogenic Granuloma, but this diagnosis was excluded around 3 weeks after the first consultation as the lesion exhibited a reduced size, brown color and a covering of mucosa.
From a microscopic point of view, these lesions exhibit individual characteristics by which they can be distinguished; TA exhibits a number of vascular lobes of varying sizes, with a predominantly solid appearance, forming tufts of fusiform or polygonal cells associated with endothelial cells, while GP is characterized by lobular vascular proliferation and circumscript proliferation of capillaries reminiscent of granulation tissue containing young fibroblasts and dense chronic inflammatory infiltrate [7]. With TA there are some well-defined vascular spaces with endothelium covering the central portion of the lobe, although the majority is located on the periphery of the lobe and has a semi-elliptical shape, and the vascular spaces can vary from poorly canalized capillaries to large vessels with thin walls and an endothelial covering of a single layer of cells [5], [9]. When GP is seen under the microscope, the lobes are made up of large vascular spaces, and of groups of small capillaries with small lumen, in addition to clusters of endothelial cells with a rounded appearance (bland appearing) and of varying sizes [10].
We also thought about the cutaneous lesions of leishmaniasis, that is an infection caused by protozoans of the genus Leishmania which is transmitted by sandflies of the genus Phlebotomus. This lesion may begin as erythematous papules which gradually increase in size, resulting in a solitary or multiple nodules, which may reach 1–2cm in diameter before ulcerating and then, after some months, heal slowly, and it is common for this disease to appear in patients who are less than 20 years old [11], [12]. This is an endemic infection in some countries, including certain regions of Brazil [12], and has become a global disease [11].
The clinical diagnosis of Leishmaniasis was ruled out because it would be necessary to observe a loose granuloma under the microscope, composed of dense lymphoplasmacytic inflammatory infiltrate, with presence of histiocytes, containing the oval organism in the interior, compatible with leishmania amastigotes, and also amastigotes in the connective tissue to confirm the diagnosis [12].
Many authors have relied on immunohistochemistry to confirm a diagnosis of tufted angioma. They have used markers such as Factor VIII-related antigen, UEA1, α1 actin, CD31, CD34, GLUT1 and D2-40 [4], [5], [9], [13]. We do not used the immunohistochemistry because the morphologic characteristics were consistent with the diagnosis of TA, and the results of immunohistochemical panel are not always specific since other vascular lesions may exhibit the same profile. Arai et al. [13] demonstrated that the D2-40 marker, which has selective reactivity to the endothelium of lymphatic channels, can be useful for differential diagnosis between tufted angioma and kaposiform hemangioendothelioma.
With relation to treatment of TA, several different approaches are employed, with surgery being the treatment of choice, particularly for small lesions [1], [3], [5]. Other treatment modalities that are used are cryotherapy, pulsed dye laser, systemic corticoid therapy and systemic interferon treatment [14]. Although some authors report recurrence of the lesion if it is not completely removed [9], there are also reports of spontaneous regression of the TA [2], justifying monitoring of asymptomatic lesions as an acceptable management strategy [1], [15].
In spite of being less frequent in the oral mucosa, TA must be considered in the differential diagnosis of oral proliferative lesions.
Acknowledgment
We acknowledge Dr. Carlos Thadeu Cerski for his help in the histological study of this case.
References
- . Tufted angioma: a report of five cases. Pediatr. Dermatol. 2002;19(5):388–393
- . Tufted angiomas: variability of the clinical morphology. Pediatr. Dermatol. 2002;19(5):349–401
- . Congenital, self-regressing tufted angioma. Arch. Dermatol. 2006;142:749–751
- . Acquired tufted angioma of the lower lip mucosa. J. Can. Dent. Assoc. 2000;66(3):1–3
- . Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa. Br. J. Dermatol. 2000;142:794–799
- . Case report: leishmaniasis of the upper lip. Oral Surg. Oral Med. Oral Pathol. 2007;104(5):659–661
- . Benign tumors and tumorlike conditions of blood vessels. In: Enzinger FM, Weiss SW editor. Soft Tissue Tumor. 2nd ed.. St Louis: Mosby; 2004;p. 489–532
- . Kasabach–Merritt syndrome. J. Cutan. Med. Surg. 2002;6(4):335–339
- . Acquired “tufted” angioma (pregressive capillary hemangioma). A distinctive clinicopathologic entity related to lobular capillary hemangioma. Am. J. Dermatopathol. 1987;9(4):292–300
- . Pitfalls in the histopathologic diagnosis of Pyogenic Granuloma. Eur. Arch. Otorhinolaryngol. 1992;249:195–200
- . Leishmaniasis: current situation and new perspectives. Comp. Immun. Microbiol. Infect. Dis. 2004;27:305–318
- . Oral leishmaniasis: a clinicopathological study of 11 cases. Oral Dis. 2007;13:335–340
- Usefulness of D2-40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma. J. Cutan. Pathol. 2006;33:492–497
- . Tufted hemangioma responding to high-dose systemic steroids: a case report and review of the literature. Clin. Exp. Dermatol. 1994;19:511–514
- . Adult-onset tufted angioma: a case report and review of literature. Cutis. 2006;78:341–345
PII: S1871-4048(08)00080-4
doi:10.1016/j.pedex.2008.12.005
© 2009 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 4 , Pages 173-176, December 2009
