the lesion has gradually enlarged with the appearance of new smaller lesions in the vicinity of the main lesion ( figure 1 ) . a biopsy revealed a vascular proliferation with round and poorly defined collections of epithelioid and spindle cells . dilated slit - like vascular spaces , resembling those of lymphatic vessels , were seen in the sclerotic stroma ( figures 24 ) . cracked irregular spaces were seen between the cells in the proliferation ( figure 5 ) . the vascular proliferation was positive for cd31 , cd34 , d2 - 40 and negative for hhv8 . the diagnosis of tufted hemangioma was established on the basis of clinical presentation , history and histopathological presentation . tufted hemangiomas are relatively rare benign vascular proliferations . although initially described in the european and japanese literature , the term  tufted hemangioma  was introduced in 1989 by jones and orkin   along with the description of the largest group of 20 patients . they usually appear during the first years of life and are congenital in up to 50% of cases . tufted hemangiomas predominantly affect males and are located on the trunk , neck or extremities . hypertrichosis and hyperhidrosis are sometimes present on the surface of plaques or surrounding the lesions . in children histopathologically the lesions are characterized by collections of vessels in small tufts with a cannonball distribution , encircled by empty cleft - like vessels and often surrounded by sclerotic dermis . tufts are composed of epithelioid and spindle cells with slit - like spaces resembling kaposi sarcoma . . widened vessels , resembling lymphatic ones , are often a source of potential diagnostic mistakes if the biopsy is taken at the periphery of the lesion . tufted hemangiomas express endothelial and lymphatic vascular markers cd31 , cd34 , vegf - a and d2 - 40 . clinical data ( hiv status , immunodeficiency , and african or mediterranean origin ) and positivity for hhv8 allow distinguish both entities with certainty . presence of spindle cells , formation of new vessels around the preexisting cutaneous structures (  promontory sign  ) , variable cellular polymorphism may help in the diagnosis of kaposi sarcoma . tufted hemangiomas tend to be located more superficially compared to superficial kaposiform hemangoenothelioma which may affect subcutis and retroperitoneum , invade the internal organs and is characterized by aggressive growth . podoplanin ( d2 - 40 ) was proposed as a useful marker for the differential diagnosis of tufted hemangioma and kaposiform hemangioendothelioma by arai et al . in their study d2 - 40 was positive in small capillaries in the kaposi sarcoma - like part of the proliferation and was negative in the widened superficial vessels in kaposiform hemangioendothelioma . in contrast , in tufted hemangiomas , d2 - 40 labeling was seen in the widened superficial vessels and was negative or patchy in the capillaries of the cannonball vessels . other studies , as well as our case report , showed inconsistency of these findings and demonstrated that d2 - 40 may also be positive in the small capillaries in tufted hemangiomas . prox1 has been recently described as a potential useful marker for the differential diagnosis of tufted hemangiomas / kaposiform hemangioepitheliomas versus infantile hemangiomas and pyogenic granuloma . it is usually positive in tufted hemangiomas/ kaposiform hemangioendotheliomas , but is not expressed in infantile hemangiomas and pyogenic granulomas . thrombocytopenia and coagulopathy in patients with tufted hemangiomas and kaposiform hemangioendotheliomas ( known as kasabach - merritt syndrome ) may be a grave consequence of both conditions . the phenomenon is explained by entrapment and adhesion of the thrombocytes to the endothelial cells of the hemangioma . if the lesion is not associated with kasabach - merritt syndrome no treatment is usually necessary .
tufted hemangiomas are relatively rare benign vascular proliferations that are congenital or appear during the first years of life 
 . herein we present an additional case of tufted hemangioma that appeared one year after birth and discuss its histopathological criteria and differential diagnosis with malignant vascular proliferations including sarcoma kaposi , angiosarcoma and kaposiform hemangioendothelioma .