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Table of Contents 
CASE REPORT
Year : 2012  |  Volume : 57  |  Issue : 3  |  Page : 228-229
Punctate vascular papules on the tongue and scrotum


1 Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication16-May-2012

Correspondence Address:
Gomathy Sethuraman
Department of Dermatology, All India Institute of Medical Sciences, New Delhi-110029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.96207

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   Abstract 

We report a 60-year-old man who presented with a 2-year history of painless red raised lesions involving the tongue and scrotum. Histopathology was suggestive of angiokeratoma. Oral angiokeratomas are most commonly found as a component of the generalized systemic disorder in Fabry's disease or fucosidosis. Our patient had isolated mucosal angiokeratomas which is very rare.


Keywords: Angiokeratoma, tongue, scrotum


How to cite this article:
Pahwa P, Meena D, Tanveer N, Sharma VK, Sethuraman G. Punctate vascular papules on the tongue and scrotum. Indian J Dermatol 2012;57:228-9

How to cite this URL:
Pahwa P, Meena D, Tanveer N, Sharma VK, Sethuraman G. Punctate vascular papules on the tongue and scrotum. Indian J Dermatol [serial online] 2012 [cited 2021 Mar 8];57:228-9. Available from: https://www.e-ijd.org/text.asp?2012/57/3/228/96207



   Introduction Top


Oral angiokeratomas are most commonly found as a component of the generalized systemic disorder in Fabry's disease or fucosidosis, where multiple angiokeratomas can be found on the skin and the oral mucosa. We report a rare case of a patient with multiple oral and scrotal angiokeratomas in the absence of a systemic disorder.


   Case Report Top


A 60-year-old man presented with a 2-year history of painless red raised lesions involving the tongue. He complained of burning sensation in the mouth in the region of the papules. He did not have any other cutaneous and/or systemic complaints. There was no preceding history of local trauma. His past medical history was unremarkable.

Clinical examination revealed multiple erythematous to slightly bluish, shiny, vascular papules ranging from 1to3 mm size, discrete to grouped on the undersurface and lateral aspects of the tongue [Figure 1] a and b. The lesions were firm on palpation and did not bleed on manipulation. The rest of the oral mucosa was normal. He had few similar papules on the scrotum [Figure 1]c. These were not blanchable. The cutaneous and systemic examination was normal. Biopsy from a vascular papule on the tongue showed acanthosis, and papillomatosis, with large, dilated spaces lined by normal-appearing endothelium which were filled with erythrocytes and organizing thrombi [Figure 1]d. A diagnosis of angiokeratoma of the tongue and scrotum was made. The patient was counseled about the benign nature of the disease after which he chose not to take any treatment.
Figure 1: (a-b) Multiple erythematous to bluish, shiny, vascular papules on the undersurface and lateral aspects of the tongue; (c) Similar vascular papules on the scrotum; (d) Acanthosis, and papillomatosis, with large, dilated spaces lined by normal-appearing endothelium which are filled with erythrocytes and organizing thrombi (H and E, × 200)

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   Discussion Top


Angiokeratoma is an asymptomatic cutaneous vascular disorder of the papillary dermis characterized by dermal vasculsar ectasia with overlying hyperkeratosis of the epidermis. [1] The pathogenesis of angiokeratoma is still unknown. It is thought to be a telangiectatic lesion arising from local injury to papillary dermal capillaries, either from trauma or venous hypertension. Five types of angiokeratoma have been described. They are (1) the generalized systemic type:angiokeratoma corporis diffusum of Fabry characterized by multiple tiny red papules in a symmetrical distribution in the bathing area; (2) angiokeratoma of Mibelli, the bilateral form occurring on the dorsal areas of the fingers and toes; (3) angiokeratoma of Fordyce, the most common angiokeratoma which occurs on the scrotum; (4) the solitary papular angiokeratoma which has a predilection for the legs; and (5) angiokeratoma circumscriptum, the multiple papular and plaque- like form. [1],[2]

Isolated oral cavity angiokeratomas are rare and few cases have been reported in literature. [3] Oral mucosal angiokeratomas have also been associated, uncommonly, with angiokeratomas of the scrotum, gastrointestinal mucosa (jejunum), or both. [4] Recently Ranjan & Mahajan have proposed a clinical classification for oral angiokeratomas, depending on the number of lesions, associated other muco-cutaneous involvement and systemic disease. [3]

Oral angiokeratomas present as single/multiple, erythematous, shiny papules, which may be studded with keratotic tops. They are firm on palpation, nontender, show telangiectatic vessels on diascopy, and bleed occasionally. The tongue, with a predilection for the dorsal aspect, appears to be the most common site for isolated oral angiokeratoma. [3] They may be mistaken for hemangiomas, lymph-hemangiomas, focal epithelial hyperplasia, or nevi. The histologic features are similar to their cutaneous counterparts; hyperkeratosis, acanthosis and dilated capillaries in the papillary dermis, partly or completely enclosed by the papillomatous epidermis. Organized or organizing thrombi are occasionally observed within the dilated capillaries. The only difference being the presence of parakeratosis in oral angiokeratomas. [5] However, in Fabry's disease and fucosidosis, swollen and vacuolated (lipid containing) endothelial cells are characteristic differentiating features. Treatment depends on the number and location of the lesions. Treatment as mentioned for angiokeratomas includes simple excision, laser ablation, and electrocautery.

 
   References Top

1.Atherton DJ. Naevi and other developmental defects. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's textbook of dermatology, 7 th ed., Vol.1. Oxford: Blackwell Science; 2004. p. 15:87-90.  Back to cited text no. 1
    
2.Karthikeyan K, Sethuraman G, Thappa DM. Angiokeratoma of the oral cavity and scrotum. J Dermatol 2000;27:131-32.  Back to cited text no. 2
[PUBMED]    
3.Ranjan N, Mahajan V K. Oral angiokeratomas: proposed clinical classification. Int J Dermatol 2009;48:778-81.  Back to cited text no. 3
    
4.Rappaport I, Shiffman MA. Multiple phlebectasia involving jejunum oral cavity, and scrotum. JAMA. 1963;185:437-40.  Back to cited text no. 4
[PUBMED]    
5.Vijaikumar M, Thappa DM, Karthikeyan K, Jayanthi S. Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol. 2003;20:180-2.  Back to cited text no. 5
    


    Figures

  [Figure 1]

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