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Table of Contents   
CASE REPORT  
Year : 2019  |  Volume : 30  |  Issue : 2  |  Page : 322-326
Isolated angiokeratomas of the tongue: A rare entity


1 Department of Oral Medicine and Radiology, Government Dental College and Hospital, Srinagar, Jammu and Kashmir, India
2 Department of Pathology, SKIMS, Srinagar, Jammu and Kashmir, India
3 Department of Pathology, GMC, Srinagar, Jammu and Kashmir, India

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Date of Web Publication29-May-2019
 

   Abstract 

Angiokeratomas consist of ectasias of dermal capillaries associated with an acanthotic and hyperkeratotic overlying epidermis. These dark red-to-purple, papular vascular anomalies can vary considerably in size, depth, and location. It is a skin disorder that rarely involves oral cavity. It can occur in localized or generalized form and is often associated with underlying metabolic disorder such as Fabry's disease and fucosidosis. It has many clinical variants with the same underlying histopathology. Mucosal involvement, including the oral cavity, is occasionally found either as a component of the systemic variety, called angiokeratoma corporis diffusum, or associated with cutaneous lesions in more locations. Isolated oral involvement seems to be rather infrequent, and only eighteen cases have been described in the world literature thus far. Isolated multiple angiokeratomas of tongue without plaque formation have been reported only four times before this. Here, we report a fifth case of isolated multiple angiokeratomas of tongue in a 16-year-old female which was confirmed by immunohistochemical pattern in consonance with a blood vessel origin, with expression of CD31, CD34, and von Willebrand factor. The lesion did not express D2-40 and CD45. No other malformation or metabolic disorder was found in the patient.

Keywords: Angiokeratoma, immuno-histochemical, isolated, oral cavity, tongue, tumor

How to cite this article:
Hamid R, Chalkoo AH, Singh I, Wani S, Bilal S. Isolated angiokeratomas of the tongue: A rare entity. Indian J Dent Res 2019;30:322-6

How to cite this URL:
Hamid R, Chalkoo AH, Singh I, Wani S, Bilal S. Isolated angiokeratomas of the tongue: A rare entity. Indian J Dent Res [serial online] 2019 [cited 2019 Jun 20];30:322-6. Available from: http://www.ijdr.in/text.asp?2019/30/2/322/259232

   Introduction Top


Angiokeratoma (AK) is a rare capillary vascular disorder, characterized clinically by asymptomatic, solitary or multiple, keratotic papules or plaques, and histologically by benign vascular ectasia of the papillary dermis.[1] It can be divided into localized and systemic form. The localized forms include (a) AK of Fordyce-usually multiple, blue-to-purple, smooth, 2–5 mm papules on the scrotum/vulva and occur rarely on the penile shaft and glans (b) AK circumscriptum – usually solitary and asymptomatic benign lesions, and usually occur on the lower extremities, may be congenital or acquired and therefore can be present at birth. They are more frequently seen in childhood and early adulthood. They are more common in females than males (3:1 ratio) and may coexist with other types of angiokeratomas and with Klippel–Trenaunay syndrome, naevus flammeus, cavernous hemangiomas, and traumatic arteriovenous fistulas. (c) Solitary angiokeratoma – presents as a small warty black/red blue papules usually after external trauma on any anatomical site but favoring lower extremities. (d) AK of Mibelli – presents in a female adolescent as dark red, warty papules on bony prominences of elbows, knees, digits, and dorsa of the hands usually after a history of trauma/chill blains.[2] The systemic form is known as AK corporis diffusum and is usually linked to some metabolic disorders, mainly Fabry's disease and also Fucosidosis. Isolated oral mucosal angiokeratomas (without cutaneous or metabolic disease); however, find no place in this description.[3] Oral mucosal involvement is commonly seen as a component of AK corporis diffusum and is very uncommon in other types of angiokeratomas.[4] Isolated angiokeratoma of oral cavity is very rare, and to the best of our knowledge, only 18 cases have been reported thus far.[1],[2],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14] Here, we report a new case and comment on the immunohistochemistry confirming its nature.


   Case Report Top


A 16-year-old female presented to Oral Medicine and Radiology Department with a chief complaint of multiple red-colored small raised lesions on the posterior dorsal surface of the tongue for the past 2 years with occasional pain and bleeding occurring with trauma. The condition started as a single raised lesion on the dorsal surface of tongue which gradually increased in number over the last two years. There was no previous history of local trauma. There was no history of similar lesions elsewhere on the body. On intraoral examination, multiple pinpoint to 4-mm diameter, dark-red to blue–black, macular and papular lesions, which did not blanch on pressure, were seen on dorsal surface of the tongue with no plaque formation [Figure 1] and [Figure 2]. They were nontender and did not bleed on manipulation. The rest of the cutaneous and systemic examination was normal. Lymphangioma and hemangioma were considered in clinical differential diagnosis. Two of these papules were excised in toto under local anesthesia [Figure 3] and the specimens were sent for microscopic examination. Histopathology of both papules had similar findings. Histopathology [Figure 4]a,[Figure 4]b,[Figure 4]c revealed several dilated variable-sized congested blood vessels in subepithelial region covered by hyperplastic, hyperkeratotic, parakeratotic and squamous epithelium. Chronic inflammatory cell infiltration around blood vessels and rete ridges are also present. Neither the epithelium nor the endothelial lining of the vessels showed any dysplasia. These features are consistent with AK. The main differential diagnosis on histopathological grounds is lymphangioma, despite hyperkeratosis is not usually seen in this tumor. To exclude this diagnosis, we performed immune-histochemical study [Figure 4]d,[Figure 4]e,[Figure 4]f with the following primary antibodies: CD31 (clone JC70A, Dako), CD34 (clone QBEnd 10, Dako), Factor VIII (clone F8/86 Dako), CD45 (Clone 4KB5, Dako), and D2-40 (clone D2-40, Dako). The endothelium of the lesion expressed CD31, CD34, and Factor VIII. It was negative for CD45 and D2-40. Definitive diagnosis was angiokeratoma. This diagnosis prompted a search for similar lesions elsewhere in the body for which she was sent for dermatologic examination. Despite a meticulous survey, none were found. The subsequent examination ruled out any systemic or biochemical abnormalities, nor did any of his family members suffer from symptomatology suggestive of a definite syndrome. Thus, a diagnosis of “Isolated Angiokeratomas of the tongue” was made. Remaining angiokeratomas are being treated in multiple settings by carbon dioxide laser therapy. None has recurred so far.
Figure 1: Discrete reddish, multiple, warty, and nonwarty papules, at the junction of anterior two-third and posterior one-third of the dorsum of the tongue

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Figure 2: Diascopy (no blanching seen)

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Figure 3: Excision of two papules under Local anesthesia

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Figure 4: Note, the prominent thickening of the mucosa overlying the proliferation of dilated vascular channels. In addition, note hyperkeratosis. A closer view shows how the dilated vessels were closely attached to the epithelium. No endothelial atypia was evidenced. (a) Panoramic view of resected specimen (H and E, ×40). (b) (H and E, ×100). (c) High-power detail of the vascular channels (H and E, ×400). (d) Panoramic view. Endothelium of the vascular formations expressed positive immunostaining for CD31 (Original magnification, ×100). (e) CD 31 positive (Original magnification, ×400). (f) No expression of CD45 was seen (Original magnification, ×400)

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   Discussion Top


The first case of angiokeratoma was reported by Mibelli[15] in 1889 on fingers and toes. Fordyce[12] in 1896 reported the first case of angiokeratoma on the scrotum. Fabry[12] in 1898 described an angiokeratoma that was associated with cardiac, renal and ocular abnormalities and in 1915[12] described a localized lesion on lower extremity, termed as AK circumscriptum. Solitary angiokeratoma as a distinct lesion was identified by Imperial and Helwig.[16] Solitary angiokeratoma without systemic involvement is rare and occurs predominantly on lower extremities but may also be found in areas such as scrotum, vulva, clitoris, and on tongue.[17] Isolated oral cavity involvement is very rare with only 18 cases reported in the world literature. First case of solitary isolated oral cavity involvement was reported by Leung et al.[5] in 1997 on buccal mucosa of an 82-year-old male. Isolated solitary as well as multiple angiokeratomas of tongue without plaque formation is very rare. The first case of isolated angiokeratoma over tongue was reported by Vijai Kumar et al.[4] Solitary angiokeratomas seems rather infrequent, with only six cases reported till date in adults [Table 1].[5],[8],[10],[11],[12],[18] Only four cases of isolated multiple angiokeratomas over tongue without plaque formation have been reported in world literature [Table 2].[2],[3],[4],[19] In our review here, we report a fifth case in a 16-year-old female child [Table 2].[2],[3],[4],[19]
Table 1: Summary of the clinical features of the reported cases (of solitary angiokeratomas)

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Table 2: Summary of the clinical features of the reported cases of isolated multiple angiokeratomas over tongue

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In adults, the isolated lesions occurred on ventral surface, tip, lateral border of tongue, buccal mucosa, and tonsillar pillar. Varshney[7] reported it to be asymptomatic; however, it may be associated with bleeding, discomfort, and cosmetic concerns. Life-threatening bleeding is not a concern; however, excessive bleeding after incisional biopsy was reported in one case having deficiency of clotting factor VIII which was an incidental finding.[18] Clinically, bleeding from angiokeratoma lesion can be mistaken for a number of lesions such as melanocytic nevus, malignant melanoma, verruca vulgaris, hemangioma, capillary aneurysm, or focal epithelial hyperplasia.[20] Histopathology of AK reveals, ectasia of vascular channels, increased blood-filled spaces associated with epithelial hyperplasia, and keratosis. Microscopic features of solitary oral angiokeratoma are similar to those arising in skin. In both sites, acanthosis and papillomatosis of squamous epithelium can be seen;[21] however, oral lesions show more hyperparakeratosis while the cutaneous lesions show hyperorthokeratosis.[11] Thrombosis of the vessels may occur resembling melanoma.[18] However, thrombosis of vessel was not found in the present case. In subepithelial tissue, chronic inflammatory infiltrate of lymphocytes and plasma cells was found.

AK is an asymptomatic, benign, and rare cutaneous vascular disorder of the papillary dermis characterized by vascular ectasia with hyperkeratosis of overlying epidermis. AKs may be either congenital or acquired. These lesions usually present at birth, but in some cases may present in early childhood and adulthood.[14] Females are commonly affected, with the male-to-female ratio being 1:3.[14] All these features were in agreement with the present case. It was an acquired angiokeratoma present in a young adolescent female.

AK is a dark, irregular lesion which bleeds occasionally. Therefore, it can be mistaken clinically for melanocytic nevus, malignant melanoma, verruca vulgaris, hemangioma, capillary aneurysm, Spitz nevus or focal epithelial hyperplasia, and lymphangioma circumscriptum.[12] The excisional biopsy with meticulous histological examination is important to confirm the diagnosis, as demonstrated in our case. The main differential diagnosis of angiokeratoma is lymphangioma circumscriptum.[12] Immunohistochemical markers for lymphatic endothelium, such as LYVE-1, has been suggested by Maria et al.[5] in case of difficulty in differentiating from lymphangioma circumscriptum. We used both vascular and lymphatic makers in our study. The endothelium of the lesion expressed CD31, CD34, and Factor VIII. It was negative for CD45 and D2-40. A thorough examination of the skin and mucous membranes must be performed to discard further lesions elsewhere and rule out an association with systemic diseases in widespread cases.

The pathogenesis of angiokeratoma is still not clear. It is thought to be a telangiectatic lesion arising from local injury to capillaries in the papillary dermis, either from trauma or venous hypertension, with secondary epithelial proliferation causing acanthosis and hyperkeratosis. It has been reported to develop overlying an arteriovenous fistula and in an area of lymphangioma circumscriptum after repeated local injuries.[22] The epidermal changes in all forms of AK are secondary.[22] Hyperkeratosis may be seen on the surface. There is associated irregular acanthosis of the epidermis with elongation of the rete ridges which partially or completely enclose the vascular channels. A collarette may be formed at the margins of the lesions and there may be thrombosis of the vessels.[22] Oral angiokeratoma has histologic features similar to those arising on the skin. However, while the cutaneous lesions show hyperorthokeratosis, the oral lesions show more hyperparakeratosis. Immunohistochemical studies have shown matrix metalloproteinase (MMP)-9 localized in the epidermis just under the horny layer, particularly in the hyperkeratotic lesions of angiokeratoma circumscriptum. The expression of MMP-9 may be related to the hyperkeratotic changes in the lesion.[22]

AK being an asymptomatic benign, vascular lesion, treatment is required when there is bleeding, discomfort, or for cosmetic purposes. Laser ablation or excision is the standard treatment. Laser ablation has been proven to be highly effective and may offer the best cosmetic outcome.[14] The other options available for removing these lesions are cryosurgery and electrodessication.[22] Treatment of isolated multiple angiokeratomas over tongue without plaque formation has not been laid down clearly in literature because only four cases have been reported thus far. Previous two reports by Vijai Kumar[4] and Bhargava[2] did not mention the form of treatment they used for isolated multiple angiokeratomas over the tongue. Two reports by Patigaroo et al.[3],[19] used both laser and surgical excisions. We also excised few papules (for biopsy and with therapeutic intention) and are using carbon dioxide laser for the rest of papules. We have achieved good results with surgical excision and laser therapy so far. Argon laser and Nd:YAG laser have been reported to effectively eliminate angiokeratoma, although there is a risk of scarring and hypopigmentation. In addition, several sessions are required with these nonablative vascular laser systems because of the hyperkeratotic epidermal reaction in these lesions which makes it difficult for the laser beam to target the superficial dermal vessels.[14] The addition of ablative laser systems such as continuous wave argon or carbon dioxide laser as used in our case results in better access because of the reduction in hyperkeratosis.[14]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
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2.
Bhargava P, Bhargava S, Mathur D, Agarwal US, Bhargava R. Angiokeratoma of tongue. Indian J Dermatol Venereol Leprol 2001;67:270.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Patigaroo SA, Khan NA, Manzoor S, Gupta N, Jain P, Shakeel M. Isolated multiple angiokeratoma of tongue – A case report and review of literature. Int J Pediatr Otorhinolaryngol Extra 2012;7:126-8.  Back to cited text no. 3
    
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Kumar MV, Thappa DM, Shanmugam S, Ratnakar C. Angiokeratoma circumscriptum of the oral cavity. Acta Derm Venereol 1998;78:472.  Back to cited text no. 4
    
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Leung CS, Jordan RC. Solitary angiokeratoma of the oral cavity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:51-3.  Back to cited text no. 5
    
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Vijaikumar M, Thappa DM, Karthikeyan K, Jayanthi S. Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol 2003;20:180-2.  Back to cited text no. 6
    
7.
Varshney S. Angiokeratoma circumscriptum of the tongue. Int J Dermatol 2005;44:886-8.  Back to cited text no. 7
    
8.
Siponen M, Penna T, Apaja-Sarkkinen M, Palatsi R, Salo T. Solitary angiokeratoma of the tongue. J Oral Pathol Med 2006;35:252-3.  Back to cited text no. 8
    
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Yildirim M, Kilinç N, Oktay MF, Topçu I. A case of solitary angiokeratoma circumscriptum of the tongue. Kulak Burun Bogaz Ihtis Derg 2007;17:333-5.  Back to cited text no. 9
    
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Ergun S, Mete O, Yeşil S, Tanyeri H. Solitary angiokeratoma of the tongue treated with diode laser. Lasers Med Sci 2009;24:123-5.  Back to cited text no. 10
    
11.
Sion-Vardy N, Manor E, Puterman M, Bodner L. Solitary angiokeratoma of the tongue. Med Oral Patol Oral Cir Bucal 2008;13:E12-4.  Back to cited text no. 11
    
12.
Fernández-Aceñero MJ, Rey Biel J, Renedo G. Solitary angiokeratoma of the tongue in adults. Rom J Morphol Embryol 2010;51:771-3.  Back to cited text no. 12
    
13.
Job AM, Aithal V, Tirumalae R. Angiokeratoma of the tongue: An unusual site. Int J Oral Health Sci 2016;6:88-91.  Back to cited text no. 13
  [Full text]  
14.
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.  Back to cited text no. 14
[PUBMED]  [Full text]  
15.
Mibelli V. Di una nuova forma di cheratosis, angiocheratoma. G Ital Mal Vener 1889;30:285-301.  Back to cited text no. 15
    
16.
Imperial R, Helwig EB. Angiokeratoma. A clinicopathological study. Arch Dermatol 1967;95:166-75.  Back to cited text no. 16
    
17.
McNeely TB. Angiokeratoma of the clitoris. Arch Pathol Lab Med 1992;116:880-1.  Back to cited text no. 17
    
18.
Fernandez-Flores A, Sanroman J. Solitary angiokeratoma of the tonsillar pillar of the oral cavity. Rom J Morphol Embryol 2009;50:115-7.  Back to cited text no. 18
    
19.
Patigaroo SA, Hashmi SF. Multiple angiokeratomas of tongue. Indian J Otolaryngol Head Neck Surg 2010;62 Suppl 2:191-3.  Back to cited text no. 19
    
20.
Schiller PI, Itin PH. Angiokeratomas: An update. Dermatology 1996;193:275-82.  Back to cited text no. 20
    
21.
Young WG, Sauk JJ Jr., Pihlstrom B, Fish AJ. Histopathology and electron and immunofluorescence microscopy of gingivitis granulomatosa associated with glossitis and cheilitis in a case of Anderson-Fabry disease. Oral Surg Oral Med Oral Pathol 1978;46:540-54.  Back to cited text no. 21
    
22.
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.  Back to cited text no. 22
    

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Correspondence Address:
Dr. Rizwan Hamid
Department of Oral Medicine and Radiology, Government Dental College and Hospital, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdr.IJDR_644_17

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