[Intravascular lymphoma revealed by generalized arborescent telangiectasia and repeated venous thrombosis]. / Thromboses veineuses récidivantes et télangiectasies arborescentes généralisées révélatrices d'un lymphome intravasculaire.

ABSTRACT

BACKGROUND: Intravascular lymphoma is a rare form of non-Hodgkin's lymphoma characterized by proliferation of lymphoid cells within the lumen of small veins, small arteries and capillaries. CASE REPORT A 79-year-old man presented with repeated superficial venous thrombosis of the lower limbs associated with diffuse telangiectasia of the trunk, upper arms and thighs but with normal epidermis. Screening for thrombophilia and neoplasm were negative. The patient subsequently developed abdominal pain, lower-limb oedema, deterioration in performance status and rapidly increasing telangiectasia with the appearance of generalized oedematous cutaneous induration. Increased LDH and anaemia were observed without other blood count anomalies. Intravascular lymphoma was diagnosed on a skin biopsy with telangiectasia and oedema. After eight courses of treatment with rituximab-CHOP the outcome was good. DISCUSSION: A few telangiectasias associated with nodules or infiltrated plaques are often a clinical manifestation of intravascular lymphoma. The dermatological presentation described here is interesting for two reasons on the one hand, telangiectasias were initially isolated in normal epidermis without any infiltration for more than one year and, on the other hand, the skin infiltration seen subsequently was very extensive and marked. Moreover, although microthrombi are frequent in small and medium-sized blood vessels, thrombosis of large vessels is rarely described in intravascular lymphoma.