A case of “purple glove syndrome”

Abstract

A 44-year-old woman, affected by Down syndrome, was hospitalized because of traumatic hip fracture. She was in treatment with valproic acid for convulsive seizures with good control. Due to a misunderstanding, the antiepileptic treatment was withdrawn and the day after the operation she fell into a coma with normal CT scan and EEG showing a non convulsive status epilepticus (NCSE) pattern. Since there was no response to intravenous valproic acid, intravenous phenytoin was started in a superficial vein of the right superior limb with a loading dose of 15 mg/kg at an infusion rate of 25 mg/min (total loading dose 900 mg with an infusion duration of 40 min) and a maintanance dosage of 100 mg TID, always in a superficial vein with an infusion rate of 25 mg/min. Partial remission of NCSE was observed but on the 3rd day of phenytoin infusion purple glove syndrome (PGS) appeared (Fig. 1). PGS is a rare serious adverse reaction to intravenous phenytoin. Its incidence ranges from 1.5 to 5.9% [1]. Its manifestation includes pain, skin discolouration, blister formation, sloughing, ulceration, and necrosis. Usually clinical manifestations are limited to the superior limbs but there are descriptions of cases with involvement of inferior limbs too (purple sock syndrome) [1]. Differential diagnoses include cellulitis, peripheral vascular disease, venous thrombosis, and collagen vascular disease. The pathogenesis is poorly understood. It has previously been considered to be a result of extravasation of the drug but PGS can occur in the apparent absence of extravasation, as in our case [1]. There is at least one description of PGS after oral subministration of a high dose of PHT [2] and it has been described also with use of fosphenytoin, a watersoluble phosphate ester pro-drug of phenytoin with pH of 8.6–9 which was developed by Pfizer to overcome infusion complications associated with phenytoin [3]. After the PGS appearance in our patient, phenytoin infusion was withdrawn and intravenous lacosamide was started with rapid improvement of PGS (Fig. 2). Unfortunately, NCSE resulted to be refractory to any other drug treatment and she died after 10 days of ICU because of hospitalacquired pneumonia.