Purpura Fulminans in Toxic Epidermal Necrolysis: Case Report and Review

Purpura fulminans (PF) and toxic epidermal necrolysis (TEN) are rare and life-threatening diseases. TEN is a notorious epidermolytic condition, most cases of which are drug induced. TEN is a more severe variant of epidermal necrolysis than Stevens-Johnson syndrome, as it affects a greater percentage of skin surface area. PF occurs in acute severe infections, deficiencies of protein C and S, and rarely, is idiopathic. PF is a thrombotic disorder of hemorrhagic cutaneous infarction and disseminated intravascular coagulation. While the two diseases are distinct in etiology, their clinical presentations can be strikingly similar. This report illustrates the clinical overlap between PF and TEN and reveals the potential for the diseases to coincide. The case of a patient with biopsy-proven TEN who developed PF was reviewed in detail. The topics of PF and TEN were searched using the MEDLINE database to investigate the relationship between the two diseases. Our case report raises diagnostic suspicion for PF in cases of TEN, particularly in patients with rapid clinical deterioration and failure of conventional management. In addition to the case presented from our institution, a similar case has been reported in which biopsy-proven PF clinically mimicked the epidermolytic condition Stevens-Johnson syndrome. These observations reflect that, although rare, conditions of epidermal necrolysis and PF may coincide more frequently than currently recognized.