Observation
A 47-year-old Caucasian woman with a 4-month history of PV presented with extensive keloid scarring at areas of prior PV erosions. Four months ago, she had been hospitalized for a severe PV flare with erosions affecting her trunk, upper and lower limbs (figure 1a). Oral, nasal and conjunctival mucosa were also involved. In addition, nail damage was noted with onycholysis, onychomadesis and necrotic paronychia (figure 1b). The Pemphigus Disease Area Index (PDAI) was 55. The diagnosis of PV was confirmed by histological and immunological investigation showing intraepidermal blistering, supra-basal epidermal acantholysis and inter-keratinocyte IgG deposits in direct immunofluorescence. ELISA was positive for anti-desmoglein 3 (120 U/ml) and anti-desmoglein 1 (180 U/ml). The erosions did not heal after 16 days of treatment with 1.5mg/kg/day of prednisone-equivalent and became extensive and hemorrhagic. The patient was apyretic. The complete blood count revealed hyperleukocytosis and hyperneutrophilia. CRP level was 386 mg/l. Two days later, a greenish malodorous discharge covered almost all the erosions (Figure 2a). Cultures grew Pseudomonas aeroginosa . Thus, the diagnosis of ecthyma gangrenosum complicating a PV was retained. A dramatic improvement of the patient’s skin erosions was observed two weeks after introducing an antibiogram-adjusted antibiotic therapy based on Tazobactam and Amikacin. After two weeks, the erosions had re-epithelialized with post-inflammatory hyperpigmentation, and the patient was discharged. We started oral steroids tapering without relapse. At the four-month follow-up, extensive keloids were noted on previous superinfected PV sites (figure 2b), for which intralesional corticosteroids were scheduled.