Objective: We report on the uncommon association between severe erythrodermic psoriasis and moyamoya angiopathy (MMA), a progressive cerebrovascular disorder characterized by steno-occlusive changes in the circle of Willis. Concomitant moyamoya and severe erythrodermic psoriasis is a rare pathology, with unknown pathogenesis. MMA with severe erythrodermic psoriasis, even in the setting of stroke, is often managed with non-curative medical intervention alone, due to concerns for surgical instability. Here we show with appropriate surgical consideration and medical management, patients can undergo curative surgical management, and remain stroke free during follow-up.
Case Report: The patient, a 52-year-old female, with refractory psoriasis, presented with neurological deficits, leading to the diagnosis of bilateral moyamoya arteriopathy. Patients with these co-existing conditions have historically only been medially managed, due to concerns for surgical instability and inadequate candidacy. A comprehensive stroke workup revealed severe stenosis in the internal carotid arteries. A two-stage surgical revascularization, including right superficial temporal artery-middle cerebral artery (STA-MCA) bypass and subsequent left STA-MCA bypass, was successfully performed. Postoperatively, the patient experienced a severe psoriasis flare, requiring meticulous management to ensure post-operative surgical stability.
Conclusions: With appropriate medical and surgical management, the patient was amenable for curative surgical intervention. The successful surgical intervention, following medical optimization of psoriasis, demonstrated efficacy in preventing future cerebral ischemia events in this challenging patient.
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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2024.107997 | DOI Listing |
Cureus
November 2024
Rheumatology, Kiran C. Patel College of Osteopathic Medicine, Nova Southeastern University, Davie, USA.
Psoriasis (PsO) is a chronic, systemic, and autoimmune dermatologic condition characterized by dry, scaly, and erythematous plaques on the skin. PsO can present in various forms, including guttate (small, round lesions commonly over the upper trunk and extremities that can be raised and scaly), inverse (smooth plaques of inflamed skin within skin folds of the groin, buttock, and breasts), pustular (white painful pustules within red inflamed blotches widespread over the body), and erythrodermic (red rash present over most of the body). Individuals with PsO can present differently, with unique symptoms and patterns on the skin.
View Article and Find Full Text PDFJ Allergy Clin Immunol
December 2024
Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, NY, USA. Electronic address:
Background: Chronic erythroderma is a potentially life-threatening condition that can be caused by a variety of diseases, but approximately 30% of cases remain idiopathic, often with insufficient treatment options.
Objective: To establish a molecular disease map of chronic idiopathic erythroderma.
Methods: We performed single-cell RNA sequencing combined with T-cell receptor sequencing of blood and skin from 5 chronic idiopathic erythroderma (CIE) patients and compared results with 8 cases of erythrodermic cutaneous T-cell lymphoma (eCTCL), 15 moderate-to-severe atopic dermatitis (AD), 10 psoriasis and 20 healthy control (HC) individuals.
Front Med (Lausanne)
November 2024
Institute of Dermatology, Chinese Academy of Medical Sciences & Peking Union Medical College, Nanjing, Jiangsu, China.
Background: Erythrodermic psoriasis (EP) is a rare but life-threatening variant of psoriasis less responsive to conventional systemic therapies (CST). Limited research exists on the management of EP with secukinumab.
Objectives: To compare the effectiveness, quality-of-life effects and safety of secukinumab versus CST in patients with EP.
The off-label use of dupilumab has proven to be an effective treatment option for severe chronic actinic dermatitis (CAD). We present the first case of CAD presenting with dupilumab-induced erythrodermic psoriasiform dermatitis. A modified, spaced dupilumab dosing regimen, combined with cyclosporine, successfully managed both conditions, offering a promising strategy for managing dupilumab adverse effects while maintaining control over chronic actinic dermatitis.
View Article and Find Full Text PDFBr J Dermatol
October 2024
Departments of Dermatology and Pediatrics, Northwestern, University Feinberg School of Medicine, Chicago, Illinois, USA.
Background: Congenital ichthyoses (CI) comprise a heterogeneous group of genetic diseases requiring lifelong treatment and having a major effect on quality of life. Conventional treatments reduce scaling and skin discomfort; however, they usually have little or no effect on erythema and pruritus. The identification of cytokine alterations in CI raised the possibility of repurposing available biologics.
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