IMERSLUND-GRASBECK-SYNDROME IN A CHINESE FAMILY WITH DISTINCT SKIN-LESIONS REFRACTORY TO VITAMIN-B-12

Two brothers in a Chinese family with selective malabsorption of vitam in B-12 associated with proteinuria (Imerslund-Grasbeck syndrome) pres ented with widespread mottled skin pigmentation, termed poikiloderma. In contrast to anaemia, this pigmentary disturbance remained unrespons ive to vitamin B,, replacement. This is different from the reported hy perpigmentation sometimes seen in vitamin B,, deficiency which is reve rsible following treatment. As far as is known, an irreversible and pe rsistent skin disorder has not been reported in this syndrome before.