epithelioid hemangioendothelioma ( ehe ) is a rare tumor of the vascular endothelium that is considered to be intermediate between hemangioma and angiosarcoma .
typically , the soft tissue is involved and , less frequently , the skin , bone , liver and lung are involved . in this report
, we describe a case of cutaneous ehe that was present since birth involving the whole of the right lower limb .
a 16-year - old female presented with painful progressive swelling and erythematous skin lesions involving the whole of her right lower limb .
she had lesions since birth and also had restricted movements but with no other systemic complaints .
an examination revealed diffuse swelling of the right lower limb , including the vulva and the lower abdomen .
there were multiple erythematous papules , nodules and plaques of varying size that were firm in consistency [ figure 1 ] .
diffuse swelling of the whole of the right limb with multiple erythematous papules , nodules and plaques on subjecting the patient for investigations , her bleeding time , clotting time , prothrombin time and complete blood count were normal .
x - ray [ figure 2 ] and computed tomography scan revealed soft tissue swelling with diffuse osteolysis of the femur , tibia and fibula .
the other differential diagnosis was klippel trenaunay syndrome , but due to the presence of osteoporotic changes in the bone , this was excluded .
x - ray showing diffuse osteolysis of the right lower limb bones compared with the normal left limb biopsy of the erythematous nodule showed nodular tumor in the dermis , which was composed of oval- to spindle - shaped epithelioid cells .
the nuclei showed a coarse chromatin pattern , and occasional mitotic figures were also noted .
the cells were strongly immunoreactive for cd34 [ figure 4 ] and factor viii - related antigen .
finally , a diagnosis of ehe was made and the patient was referred to the vascular surgery department for further management .
hematoxylin and eosin showing multiple vascular spaces with red blood cells ( 40 ) cd 34 antigen positivity ( 40 )
ehe has also been reported in the liver , bone , gingiva , mediastinum and lung .
ehe is most commonly located on the extremities , and tumor is evenly distributed through all the adult age groups .
the neoplasm usually presents as solitary , rarely multiple , slightly painful erythematous papules , nodules , plaque and nonhealing ulcer .
microscopic examination reveals aggregates of oval to polygonal cells with abundant eosinophilic cytoplasm and round nucleus . the nuclear chromatin pattern is vesicular and frequent prominent nucleoli are present .
the more commonly used antisera are factor viii - related antigen , cd 31 , cd34 and ulex europeus lectin .
our case is unusual because of its presence since birth and because of its location in the dermis and involving the underlying bone with diffuse osteolysis .
a case of cutaneous ehe with underlying bone involvement was described by malane , sau , benson in 1992 .
the prognosis of ehe is uncertain as the mortality rate for ehe of the liver is 35% and lung is 65% .
treatment should be limited to simple surgical excision , provided the possibility of an underlying bone lesion has been excluded .
 epithelioid hemangioendothelioma is an intermediate - grade vascular tumor arising from the vascular endothelium , which usually arises in soft tissue , and skin involvement is extremely rare . 
 we report a case that presented with primary cutaneous tumor involving the whole limb and was present since birth . 
Introduction
Case Report
Discussion