A previously healthy 43-year-old female presented to the emergency department with a generalized rash for 1 month and intermittent fevers. The patient's only reported new exposure consisted of elective intravenous vitamin infusions consisting of glutathione, ascorbic acid, selenium, zinc, and vitamin B complex obtained at a local medi-spa approximately 1 week prior to skin eruptions. Numerous outside dermatology evaluations had been initially pursued with inconclusive biopsies and the patient had been unsuccessfully treated with topical and systemic steroids, doxycycline, cephalexin, and oral cyclosporine for presumed pustular psoriasis.
Upon transfer to our institution, the patient presented with confluent erythematous, necrotic papules and plaques with weeping extensive ulcerations and haemorrhagic crusting on her face, abdomen, extremities, and back (Figure 1a). On her bilateral lower extremities there were violaceous papules, purpuric, umbilicated, haemorrhagic bullae, and numerous scalloped confluent ulcerations (Figure 1b). The patient reported associated discomfort and pain. The lesions covered approximately 80% of the patient's body, including her external genitalia. There was no ocular, oral, or vaginal involvement at the time of admission. Nikolsky sign was negative.
The patient's labs showed elevated WBC (10.9 × 103/μL; normal 5–10 × 103/μL), electrolyte imbalance (sodium 123 mEq/L, normal 136–145 mEq/L; chloride 93 mEq/L, normal 95–105 mEq/L), hypocalcaemia (calcium 7.6 mg/dL, normal 8.4–10.2 mg/dL), transaminitis (ALT 230 U/L, normal 8–40 U/L; AST 83 U/L, normal 8–40 U/L), and hypoalbuminemia (2.5 g/dL, normal 3.5–5.5 g/dL). Swabs obtained from intact vesicles and ulcerations were negative for herpes simplex virus and varicella. A wound culture of pustule was positive for many Enterobacter species, Escherichia coli, and Staphylococcus lugdunensis. Urine culture was positive for E. coli and Enterococcus. Blood culture showed no growth. Direct immunofluorescence performed at the same time as the original outside biopsy suspicious for pustular psoriasis was negative. Serologies were negative for HIV, hepatitis B and C, and rheumatoid factor. Flow cytometric analysis showed no evidence of lymphoproliferative disorder.
The patient was continued on cyclosporine 4 mg/kg/day twice daily and started on intravenous fluids, vancomycin, piperacillin and tazobactam, mupirocin 2% ointment twice daily to eroded areas, and triamcinolone 0.1% ointment twice daily to erythematous lesions.
Despite therapy, her lesions further evolved into confluent pustulovesicles, eroded papules, plaques, crust, haemorrhagic bullae, and macerated, eroded plaques in the axillae and inframammary regions (Figure 2). Additionally, she developed erosions on her dorsal tongue, clitoral hood, and labia minora. Given the progression, the patient was transferred to the burn unit.
A repeat biopsy revealed interface vesicular dermatitis with epidermal necrosis which was consistent with PLEVA (Figure 3a, b). Repeat direct immunofluorescence showed linear C3 and IgA at the basement membrane zone which was attributed to linear IgA deposits secondary to vancomycin, or more likely, nonspecific findings sometimes reported in FUMHD patients.
A repeat biopsy revealed interface vesicular dermatitis with epidermal necrosis. Repeat direct immunofluorescence showed linear C3 and IgA at the basement membrane zone which was attributed to linear IgA deposits secondary to vancomycin.
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