The heart in lentiginosis.

Three children with classical lentiginosis and cardiac involvement are described.Full investigation and two subsequent necropsies confirmed that the heart disease was due to severe hypertrophic obstructive cardiomyopathy.Lentiginosis or hyperactivity of the melano- cytes in skin presents in children as multiple small pigmented spots and thus may be erroneously diagnosed as von Reckling- hausen's disease (Moynahan, i962), but there are no neurofibromata and there may be other associated systemic disturbances such as somatic infantilism, short stature, and car- diac abnormalities.Walther, Polansky, and Grots (I966) described a family with lenti- ginosis, abnormal electrocardiograms, and systolic murmurs; they suggested that the cardiac abnormalities were due to a familial disorder of the conducting system, as did Matthews (I968).A patient with typical lentiginosis was fully investigated in the National Heart Hospital in I967 and was shown to have classical hypertrophic cardio- myopathy; these findings were reported by Moynahan and Polani (I968) and Moynahan (1970).Recently, at the Dermatological Section ofthe Royal Society of Medicine (Moyna- han, 1970) it was suggested by one of us that patients with lentiginosis should be regularly assessed by the cardiologist, and even in the presence of a near normal electrocardiogram and normal findings on right heart catheteriza- tion, a guarded long-term prognosis should be given as these findings do not exclude the presence or subsequent development of car- diomyopathy.The purpose of this study is to document the cardiological findings as not much attention has been given to this in previous communications. Case reportsCase I A boy who died at age I6 years was the ninth of I2 children.The birthweight was 3854 g.He fed poorly, did not gain weight, and was sub- ject to sweating and throbbing attacks for the first two years of life.A murmur was heard at i year, and because he only weighed 6-8 kg, he was