SUMMARY Cutis marmorata telangiectatica congenita was observed in two adult sisters. The condition appeared at birth and did not change much with age. In one sister the condition was accompanied by hypertension, acrocyanosis and ulceration of the big toe. On the basis of a comparison of the symptoms in patients and their relatives, the authors assume that Van Lohuizen syndrome is a dominantly inherited genetic disorder with low penetrance and great intrafamilial variability.

A patient suffering from an advanced and inoperable gastric carcinoma developed an unusual paraneoplastic syndrome, consisting of intensive pruritus, acanthosis nigricans, sign of Leser-Trélat (seborrhoic keratoses and freckles), warty and papillomatous excrescences, palmoplantar hyperkeratosis and florid papillomatous and granular lesions of the mouth mucosa. The whole skin of the patient was involved. The authors believe this disorder to be generalized acanthosis nigricans. As a cause they suggest the possibility of a growth-stimulating factor, produced by the tumor cells. The relationship of acanthosis nigricans with the sign of Leser-Trélat is discussed.

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