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 .
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 .
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 ] .
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 1d ] .
the patient was counseled about the benign nature of the disease after which he chose not to take any treatment .
( 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 )
angiokeratoma is an asymptomatic cutaneous vascular disorder of the papillary dermis characterized by dermal vasculsar ectasia with overlying hyperkeratosis of the epidermis .
it is thought to be a telangiectatic lesion arising from local injury to papillary dermal capillaries , either from trauma or venous hypertension .
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 .
oral mucosal angiokeratomas have also been associated , uncommonly , with angiokeratomas of the scrotum , gastrointestinal mucosa ( jejunum ) , or both .
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 .
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
. 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 .
however , in fabry 's disease and fucosidosis , swollen and vacuolated ( lipid containing ) endothelial cells are characteristic differentiating features .
 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 . 
Introduction
Case Report
Discussion