Discussion
Three main factors of keloids are known: infection, deep injury and mechanical trauma [5]. So far, only four cases of keloid scars following pemphigus have been reported [1–4]. Gupta et al. reported a case of uninfected PV erosions that healed with keloids despite high dose of systemic steroids [2]. Shivaswamy et al. described keloids in a patient whose pemphigus was treated, inter alia , with cyclosporin. The onset of keloids in this patient was attributed to cyclosporin [3]. In the latest two cases, such as in our patient, extensive keloids appeared in patients whose pemphigus had been superinfected. Authors suggested that secondary infection induced keloids via dermal damage. However, the paucity of cases of keloids following PV flares contrasts with the frequency of superinfections which are common in pemphigus, suggesting the presence of other unknown factors [1,4].
Our case is notable due to the severity of the PV flare which was refractory to conventional treatment and then complicated by ecthyma gangrenosum. Re-epithelialization took more than one month and ended with extensive keloids after three months. It is reasonable that superinfection led to deep intradermal inflammation and keloid formation. In fact, keloids occurred in previously superinfected PV areas. In this context, there are reports of keloid onset following herpetic infections, with the keloids involving previously infected areas [6]. An alternative hypothesis is that erosions in our patient took exceptionally long to heal, which maintained a long inflammation and scarring process with a chronic fibroblast activation. This does not exclude the potential role of apoptotic cells as well as increased levels of tumor necrosis factor-alpha, found in PV, that may contribute to the dysregulation of fibroblasts, and lead to keloids in susceptible patients [7]. Intralesional steroids are generally proposed in patients with keloids. Yet, our patient is under high systemic doses of steroids, making this alternative debatable as we can opt for compression clothing and active monitoring.