A long-acting
somatostatin analog,
SMS 201-995, is now available to treat the hormonal manifestations of
islet cell tumors. We report its use in a patient with a metastatic
glucagonoma refractory to conventional
therapy. This patient, who was severely disabled by the
rash of
necrolytic migratory erythema and
brittle diabetes mellitus, allowed us to evaluate the therapeutic efficacy of
SMS 201-995 and to gain insight into the origin of the
rash.
SMS 201-995 was administered subcutaneously (.05 mg twice a day). The
rash improved markedly within 48 hours and was completely resolved within 1 week of treatment.
Insulin requirements decreased from 90 U/day to zero during the first week of treatment. Corresponding to improvement in clinical symptoms circulating
glucagon levels showed a marked decrease. There was no substantial change in plasma or urinary levels of
zinc or in plasma
amino acid levels. When
SMS 201-995 was stopped, the
rash recurred within 36 hours and it improved within 48 hours of readministration. The
rash and diabetes have remained well controlled during 8 months of
therapy but no change in
tumor size has been seen on CT scan. The rapid changes in the
rash related to the administration of
SMS 201-995 indicate that the pathogenesis of
necrolytic migratory erythema is probably due to circulating hyperglucagonemia or some other hormonal substance produced by the
tumor.