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| CASE REPORT |
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| Year : 2016 | Volume
: 6
| Issue : 2 | Page : 88-91 |
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Angiokeratoma of the tongue: An unusual site
Anupa Mary Job1, Vijay Aithal1, Rajalakshmi Tirumalae2
1 Department of Dermatology, St. John's Medical College, Bengaluru, Karnataka, India
2 Department of Pathology, St. John's Medical College, Bengaluru, Karnataka, India
| Date of Web Publication | 13-Feb-2017 |
Correspondence Address:
Anupa Mary Job
Department of Dermatology, St. John's Medical College, Bengaluru, Karnataka
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/2231-6027.199984
Angiokeratomas are a group of disorders characterized by ectatic dermal capillaries with a prominent epidermal response in the form of hyperkeratosis, occurring in clinically distinct conditions. Solitary angiokeratoma of tongue without association with systemic conditions such as Fabry's disease and fucosidosis is very rare. We present a case of solitary angiokeratoma of tongue in a 12-year-old otherwise healthy male with no evidence of systemic associations. Keywords: Angiokeratoma, oral cavity, tongue
How to cite this article:
Job AM, Aithal V, Tirumalae R. Angiokeratoma of the tongue: An unusual site. Int J Oral Health Sci 2016;6:88-91 |
| Introduction | |  |
“Angiokeratoma” is a term used to collectively describe clinically distinct conditions which share a clinical presentation with asymptomatic hyperkeratotic cutaneous vascular lesions and a histological combination of superficial dermal vascular ectasia with overlying hyperkeratosis.[1] It is found in 0.16% of the general population. They can be classified as localized and generalized systemic types. The localized forms are further subdivided into four types: (a) solitary angiokeratoma of the legs, (b) fordyce type, (c) angiokeratoma of mibelli, and (d) angiokeratoma circumscriptum.[2] The generalized systemic variant of angiokeratoma is commonly found in association with metabolic disorders such as Fabry's disease and fucosidosis. Oral angiokeratomas are rare and when found is seen as a part of a more generalized cutaneous disease. Solitary angiokeratoma of oral mucosa with no systemic involvement is rarer still, with about 17 cases reported thus far in world literature.[3],[4],[5],[6]
In the setting of rarity of this condition occurring as an isolated entity in the absence of inborn errors of metabolisms such as Fabry's disease, we report a case of isolated angiokeratoma of tongue in a 12-year-old, otherwise healthy male.
| Case Report | | |
A 12-year-old male presented with asymptomatic reddish raised lesions on the tongue since birth with occasional pain and bleeding occurring with trauma. Examination of the oral cavity revealed two discrete reddish-blue, nonwarty papules, with an average size of 1 cm × 1 cm, at the junction of anterior two-third and posterior one-third of the dorsum of the tongue [Figure 1]. Diascopy showed telangiectasia. There were no similar lesions noted elsewhere on the body. General physical examination and systemic examination was normal. | Figure 1: Discrete reddish-blue, nonwarty papules, at the junction of anterior two-third and posterior one-third of the dorsum of the tongue
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Based on the above clinical picture, a differential diagnoses of hemangioma, lymphangioma circumscriptum, angiokeratoma, and Kaposi's sarcoma were considered. The lesion was completely excised under general anesthesia and sent for histopathological study. Histopathology showed fibro-muscular tissue covered by keratinized stratified squamous epithelium with acanthosis and papillomatosis. Foci of dilated blood vessels were seen underneath the epithelium [Figure 2],[Figure 3]. These features were found to be consistent with angiokeratoma. | Figure 2: Fibro-muscular tissue covered by keratinised stratified squamous epithelium with acanthosis and papillomatosis (H and E, ×4)
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 | Figure 3: Foci of ectatic blood vessels seen beneath the epithelium (H and E, ×20)
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The absence of dilated lymphatics, proliferation of endothelium and vascular channels and prominent hemorrhages in the dermis, ruled out the other close differentials initially considered, namely, lymphangioma circumscriptum, hemangioma, and Kaposi's sarcoma.
Complete blood count, renal function tests, echocardiogram and ultrasound abdomen, antinuclear antibody, to rule out systemic associations such as inborn errors of metabolism were done and revealed no contributory findings. Ophthalmic, neurological evaluation was within normal limits. Specific tests such as α glucosidase A levels, mutation analysis were considered, however, deferred in view of no clinical evidence of systemic involvement and monetary constraints. Thus, a final diagnosis of angiokeratoma of the tongue was derived.
| Discussion | | |
The term “angiokeratoma” is derived from a Greek word which translated to a vessel, horn or a tumor.[1] The terminology was first adopted by Mibelli in 1891[2] who described it as a rare cutaneous vascular disorder of the papillary dermis characterized by vascular ectasia with overlying epidermal hyperkeratosis.[3] The clinical variants of angiokeratoma are Mibelli type acral angiokeratomas, Fordyce type occurring on scrotal skin, solitary and multiple type, Angiokeratoma circumscriptum (a nevoid hamartoma), and angiokeratoma corporis diffusum (associated with inborn errors of metabolism).
Mucosal involvement, including the oral cavity, has been described in the systemic forms as well as a component of localized type rarely.[3] Solitary case of angiokeratoma of the oral mucosa without lesions elsewhere is very rare.
Ranjan and Mahajan [4] classified oral mucosal angiokeratoma as described in [Table 1].
The pathogenesis of angiokeratoma is ambiguous. Localized angiokeratoma may be seen in a setting of acute or chronic trauma, chilblains, high venous pressure, or nevoid or vascular malformation. Several theories have been proposed in literature which includes the occurrence of lesions secondary to a local trauma, subcutaneous hematomas, tissue hypoxia,[5] as a result of high venous pressure or as a part of vascular malformation. The primary event is vascular ectasia within the papillary dermis. Epidermal changes seem to be a secondary reaction. It has been speculated that the increased cell proliferation on the surface of vascular malformations and the close location of the vascular spaces with the epidermis in angiokeratoma could explain the reactive epidermal growth. In hyperkeratotic forms of angiokeratoma circumscriptum, metalloproteinase 9 may be seen just beneath the stratum corneum. Oral involvement may be seen in a setting of angiokeratoma corporis diffusum. Anderson-Fabry disease is an X-linked lysosomal disorder marked by a deficiency of α-galactosidase deficiency. Apart from oral involvement, it may be associated with cutaneous angiokeratomas distributed symmetrically in a bathing suit pattern. Fucosidosis is other lipid storage disorders in which oral angiokeratomas are seen.[7]
The main differential diagnosis considered in this case was verrucous hemangioma and lymphangioma circumscriptum. Verrucous hemangiomas are uncommon congenital localized vascular malformations, clinically mimicking angiokeratoma, classically distributed in the extremities and histopathologically characterized by dilated capillaries along with the presence of inflammatory cells and fibrosis in the upper dermis. The lesion typically extends into the subcutaneous fat. Additional immunohistochemical staining with endothelial markers such as CD31 helps to confirm the diagnosis. Lymphangioma circumscriptum are uncommon hamartomatous malformations of the lymphatic system clinically characterized by grouped translucent thin-walled vesicles up to 5 mm diameter, containing clear lymphatic fluid. They can involve the skin and mucous membranes almost equally. Histopathologically, the lesions are characterized by numerous dilated lymphatic channels in the upper dermis, usually thick-walled containing smooth muscle, with the lumen composed of lymphatic fluid, red blood cells, and lymphocytes. The interstitium typically shows features of fibroplasias.
The treatment options for angiokeratoma depend on the site and size of the lesions. Simple excision and closure is usually considered in case of few lesions. Cryotherapy, electrocautery, and radio-frequency are the modalities currently preferred for multiple lesions.[8] Kar and Gupta described theCO2 laser and pulse dye laser in angiokeratoma of tongue and found 75% improvement with it.[3]
Our patient underwent complete excision of the lesions with no complications noted on follow-up till date. This case is reported in view of the rarity of occurrence of solitary angiokeratoma of the oral cavity in the absence of systemic diseases such as inborn errors of metabolism.
| Conclusion | | |
Angiokeratomas are cutaneous vascular disorders of papillary dermis characterized by vascular ectasia and overlying epidermal hyperkeratosis. Oral angiokeratomas are rare and are seen usually in association with storage disorders such as Fabry's disease and fucosidosis. We have presented a case of solitary angiokeratoma of tongue occurring in an otherwise healthy 12-year-old adult male since birth.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Lu YY, Lu CC, Wu CS, Wu CH. Familial angiokeratoma corporis diffusum without identified enzyme defect. Indian J Dermatol Venereol Leprol 2015;81:46-9.  [ PUBMED]  |
| 2. | Imperial R, Hebwig EB. Angiokeratoma, a clinicopathological study. Arch Dermatol 1967;95:166-175. |
| 3. | Kar HK, Gupta L. A case of angiokeratoma circumscriptum of the tongue: response with carbon dioxide and pulsed dye laser. J Cutan Aesthet Surg 2011;4:205-7. [ PUBMED] |
| 4. | Ranjan N, Mahajan VK. Oral angiokeratomas: proposed clinical classification. Int J Dermatol 2009;48:778-81. |
| 5. | Kamoona B, Kazandjieva J, Nikolova A, Bardarov E, Marina S. Angiokeratoma circumscriptum: Successful treatment with a new technology. Acta medica bulgarica 2012;39:49. |
| 6. | Karthikeyan K, Sethuraman G, Thappa DM. Angiokeratoma of the oral cavity and scrotum. J Dermatol 2000;27:131-2. |
| 7. | Yaqoob N, Ahsan A, Ahmed Z, Husain A, Ahmed R, Kayani N, et al. Angiokeratoma of tongue: a series of 14 cases. J Pak Med Assoc 2006;56:285-7. |
| 8. | Oezdemir M, Baysal I, Engin B, Oezdemir S. Treatment of angiokeratoma of fordyce with long-pulse neodymium-doped yttrium aluminium garnet laser. Dermatol Surg 2009;35:92-7. |
[Figure 1], [Figure 2], [Figure 3]
[Table 1]
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