Erythema necrolyticum migrans (ENM) usually presents as a cutaneous paraneoplastic phenomenon which is in most cases associated with a glucagon-producing tumour. Here it is for the first time described as a side-effect of glucagon treatment in persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI). In both patients, the skin lesions disappeared after discontinuation of glucagon administration. In the first child the erythema resolved without scarring within 10 days after glucagon was substituted with other medication while in the second patient healing followed subtotal pancreatectomy which rendered glucagon infusion unnecessary. Initially the clinical resemblance to atopic dermatitis is prone to cause diagnostic errors, especially in this age group.
Conclusion: erythema necrolyticum migrans should be considered as a differential diagnosis in patients who develop erythematosquamous skin lesions under glucagon treatment.