Adult-onset Still’s disease following severe acute respiratory syndrome coronavirus 2 vaccination presenting with persistent psoriasis-like dermatitis: A case report
Journal
Dermatologica Sinica
Series/Report No.
Dermatologica Sinica
Journal Volume
42
Journal Issue
2
Start Page
164-165
ISSN
1027-8117
2223-330X
Date Issued
2024-01-01
Author(s)
Hsu, Chia-Jung
DOI
10.4103/ds.DS-D-23-00151
Abstract
Dear Editor, Adult-onset Still’s disease (AOSD) is the adult form of systemic juvenile idiopathic arthritis, which is a rare autoinflammatory disorder affecting innate immunity. Several studies have reported flare-ups or the onset of AOSD after a COVID-19 infection or COVID-19 vaccination.[1] In this report, we describe a case of a patient with AOSD who presented with psoriasis-like dermatitis. Forty-five days following his third dose of COVID-19 vaccination (a Moderna mRNA-1273 booster), a 43-year-old male presented with remittent fever, reaching up to 39°C, for 2 weeks. This was followed by a sore throat, myalgia, abdominal pain, watery diarrhea, and a generalized, evanescent, slightly pruritic rash. He had received his previous two doses of COVID-19 vaccine, both ChAdOx1-S/nCoV-19 vaccine (Vaxzevria/Covishield, AstraZeneca) about 9 months and 6 months before this admission, respectively. Laboratory data disclosed abnormal liver function (ALT: 270 U/L), a high C-reactive protein (CRP) level, and a high ferritin level (19,559 ng/mL). All autoimmune serologic profiles, including antinuclear antibody, rheumatoid factor, and lupus anticoagulant, were negative. A diagnosis of AOSD was made according to Yamaguchi’s criteria after excluding other autoimmune diseases, infectious diseases, and malignancies.[2] His symptoms subsided soon after the initiation of systemic steroid treatment (prednisolone 20 mg/day; 0.3 mg/kg/day) with a gradual tapering plan and the addition of methotrexate at 10 mg per week. However, 7 months later, he experienced a sudden onset of several well-defined, elevated salmon-pink scaly plaques on his forehead, neck, upper extremities, and knees [Figure 1a and b]. The pathology report of the lesional skin suggested psoriasis. On close inspection, scattered and grouped dyskeratotic cells in the upper epidermis were present [Figure 2]. In addition, there were focal parakeratosis and a superficial perivascular infiltrate predominantly consisting of lymphocytes, plasma cells, and a few neutrophils. Laboratory data revealed high interleukin (IL)-6 level (43.8 pg/mL), high ferritin level (>40000.00 ng/mL), and high CRP level. A flare-up of AOSD with persistent psoriasis-like dermatitis was diagnosed.Figure 1: Psoriasiform dermatitis on the trunk and extremities (a and b); flagellate dermatitis presents as pruritic, erythematous, linear streaks on the trunk and proximal extremities (c).Figure 2: Grouped dyskeratotic keratinocytes in the upper epidermis.His skin lesions subsided 2 weeks after up-titrating the methotrexate dose to 15 mg per week and administering systemic steroids with methylprednisolone at 40 mg/day. However, a high spiking fever recurred, and another episode of flagellate dermatitis [Figure 1c] with a recurrent high ferritin level (>40000.00 ng/mL) was also documented. Systemic pulse steroid with methylprednisolone 1000 mg for 3 consecutive days was used for disease control. Subsequently, his fever curve and skin rash subsided and the ferritin level began to decrease. DISCUSSION AOSD is an autoinflammatory systemic disorder characterized by spiking fever, arthritis, evanescent rash, and hyperferritinemia. The typical evanescent rash is considered a major diagnostic criterion with high sensitivity and specificity. There are several nonclassical morphologic patterns of AOSD, such as persistent pruritic papules and plaques, urticarial plaques, lichenoid papules, prurigo pigmentosa-like, and dermatomyositis-like skin eruption.[2] These persistent eruptions are characterized by distinctive pathologic features, such as dyskeratotic keratinocyte in the upper half of the epidermis. Notably, these eruptions are more commonly observed in Asians and have been associated with a worse prognosis.[3,4] A comprehensive study of AOSD after COVID-19 vaccination reveals that most cases occurred following mRNA vaccination, particularly after the first dose, with the time to onset ranging from 0 days to 6 months following vaccination.[1] While 80% of the cases occurred within the first 3 weeks of vaccination, our case exhibits a relatively late onset compared to previously reported cases. The exact cause of the late onset of AOSD in our case is unknown, but stronger immunostimulation or booster effect from his first mRNA vaccination is one possible explanation. AOSD is believed to be triggered by various extrinsic factors, including infections and vaccinations. The nature infection of the COVID-19 infection increases the risk of AOSD through the recognition of spike proteins as pathogen-associated molecular patterns by pattern recognition receptors (PRRs). Similarly, COVID-19 vaccination stimulates innate immunity through PRRs.[5] These processes further activate macrophages, leading to the release of ferritin and NOD-like receptor protein 3 inflammasomes, which subsequently promote the release of IL-1b, IL-6, and tumor necrosis factor-a.[6] All of these cytokines contribute to the inflammatory cascade. A possible pathogenesis involves immune cross-reactivity due to structural homology between self-proteins and pathogens. This is the first case of AOSD reported after severe acute respiratory syndrome coronavirus 2 vaccination, presenting with psoriasis-like dermatitis during a flare-up period. New onset and flare-ups of psoriasis have frequently been reported following COVID-19 vaccination.[7,8] The accurate differential diagnosis of psoriasis and persistent psoriasis-like dermatitis in AOSD is crucial for optimal management. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Data availability statement The dataset and current study are publicly available. Financial support and sponsorship Nil. Conflicts of interest Prof. Tsen-Fang Tsai, an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.
SDGs
Publisher
Medknow
Type
letter