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.