Pustular psoriasis triggered by Mpox vaccine

[Fred]

In this case-report, we describe an Human immunodeficiency virus positive patient with generalised pustular psoriasis (GPP) possible triggered by monkeypox vaccination and eventually successfully treated with spesolimab, an interleukin-36 inhibitor.

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A man in his thirties with well-controlled Human immunodeficiency virus (HIV) infection (CD4 cell count of 841 cells/mm3) and without previous skin disease developed a rash 7 days after monkeypox vaccination (Imvanex, Lot. No.: FDP00007). His regular medications (abacavir/dolutegravir/lamivudine, desloratadine and melatonin) were unchanged for years. The rash gradually worsened with pustules, fever and systemic symptoms, and he was admitted to the Infectious diseases department. Initially, Herpes simplex virus (HSV) type 2 was detected from the skin, and he was treated with intravenous acyclovir and dicloxacillin. Repeated blood cultures, viral and bacterial skin swabs were negative. After 1 week of treatment, there was no improvement in either the rash or his general condition and he was transferred to the department of Dermatology.

Upon admission, he presented with a generalised erythematous rash with widespread pustules. As the fever and leukocytosis were interpreted to be secondary to the skin inflammation, antibiotics and antiviral therapy were paused and he was treated with intravenous fluids, acetaminophen and topical hydrocortisone butyrate 0.1%. However, his condition rapidly declined with persistent fever above 39°C, tachycardia, hypotension, and an elevated respiratory rate.

Clinically, we first considered AGEP possibly triggered by monkeypox vaccination. The morphological picture could not distinguish between AGEP and GPP and as the rash did not improve, we considered GPP as a differential diagnosis. Treatment with acitretin 0.75 mg/kg was initiated but discontinued after 2 days due to a threefold elevation in liver enzymes.

A comprehensive reassessment for infections came out negative, also test for serum anti-HSV immunoglobulin (Ig) M. A chest & abdomen computer axial tomography revealed basal lung infiltrates and hepatomegaly. To cover Gram-negative bacteria causing a nosocomial pneumonia, he received intravenous treatment with a third-generation cephalosporin, and his condition improved rapidly concomitantly with resolution of the rash.

Due to a relapse, he was readmitted 10 days later. Recalcitrant AGEP was considered, and a second biopsy was taken. As soon as his liver enzymes were normalised, acitretin was re-initiated in a lower dose (0.35 mg/kg). In addition, he was given oral cyclosporin 4-5 mg/kg. His rash and overall condition responded well to this combined treatment. The second biopsy showed similar findings, but the number of eosinophils was substantially decreased. Four weeks later, his renal function deteriorated (serum creatinine 189 µmol/L, ref 60–105 µmol/L). His blood lipids also increased considerably (total cholesterol 10 mmol/L (ref 3.3-6.9 mmol/L) prompting an alternative treatment strategy.

Due to the lack of clinical improvement, as we would have expected with AGEP, we finally considered GPP as the most likely diagnosis. A third biopsy showed similar findings as the previous biopsies; the epidermis was acanthotic with increased basal proliferation, pronounced hypogranulosis and compact parakeratosis with small collections of neutrophilic granulocytes. In the dermal papillae small, tortuous capillaries were seen.

Intravenous treatment with spesolimab was administered approximately 4 months after onset of symptoms. Encouragingly, this led to rapid clearance of his rash and clinical improvement within the first few days. Clinical scorings revealed a GPP Physician Global Assessment (GPPGA) score of 3 and Dematology Life Quality Index (DLQI) of 30 before treatment. One week after treatment, GPPGA was reduced to 1 and DLQI to 4. The treatment had no impact on regular CD4 cell counts, or on HIV-, Cytomegalovirus- and EBV-DNA level quantification. He is still in remission 8 months after treatment, back to work with a GPPGA score and DLQI of 0.

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*Funding: Early view funding unknown