An unusual nasal alar lesion

Abstract

preconised. At the age of one month, the infant abruptly presented with serious bleeding from the central ulceration that caused a drop in haemoglobin and haemorrhagic shock. Urgent surgical excision was performed with favourable outcome. Histopathologic examination showed proliferation of multiple lobules of capillaries with haemorrhagic necrosis in contact with an ulcerated epidermis. Immunostaining for GLUT1 was negative in endothelial cells.Congenital haemangiomas are fast-flow vascular tumours that proliferate fully in utero, and can be diagnosed from the 4th month of pregnancy, using antenatal ultrasonography. 1 Congenital haemangiomas are usually benign tumours with good prognosis. Untreated, RICHs involute rapidly, usually in the first 14 months of life, often leaving an area of atrophic or excess skin. However, serious complications can occur such as cardiac failure, coagulopathy or thrombocytopenia. 2 We report a rare case of ulcerated RICH complicated with haemorrhagic shock. To the best of our knowledge, there are only six other similar cases reported in the English literature. [2][3][4][5] Severe haemorrhages seem to complicate congenital haemangiomas more than infant haemangiomas. This can be explained by the presence of larger and more superficial vessels in congenital haemangiomas making small cutaneous ulceration more susceptible to cause serious bleeding 5 . One other risk factor for severe haemorrhages is RICH subtype. Indeed, our case and in 5 of the 6 other cases reported were of RICH subtype. Therefore, the Doppler ultrasound or magnetic resonance imaging is recommended in the case of ulcerated RICHs to assess the size and depth of the vessels involved and to evaluate the risk of haemorrhage. Due to their favourable prognosis and rapid involution, RICH seldom requires treatment. In case of life-threatening bleeding congenital haemangiomas, treatment must be urgent. It consists of selective vascular embolisation or surgical excision. Topic tranexamic acid is also proposed to treat bleeding episodes complicating congenital haemangiomas. 4 For our patient, we opted for surgery. Evolution was favourable.In conclusion, severe haemorrhage is a rare but serious complication of RICH. A close follow-up is recommended for cases of ulcerated congenital haemangiomas.