Recalcitrant Erythrodermic Psoriasis Complicated by Septic Shock and Mycotic Aneurysm: A Call for Treatment Guidelines

August 2024 | Volume 23 | Issue 8 | 7751 | Copyright © August 2024


Published online July 11, 2024

William Mark Richardson MD,a Justin W. Marson MD,b Samantha Shwe MD,c Cameron A. Harding MDd

aRenaissance School of Medicine, Stony Brook University, Stony Brook, NY
bDepartment of Dermatology, SUNY Downstate Health Sciences University, Brooklyn, NY
cDepartment of Dermatology, University of California, Davis Health, Sacramento, CA
dDepartment of Medicine, University of California, Irvine Medical Center, Orange, CA

Abstract
Erythroderma is characterized by diffuse erythema and scale covering over 90% body surface area that can affect individuals with inflammatory dermatoses such as psoriasis. Complications of erythrodermic psoriasis include infection and cardiovascular compromise. Here we present a case of a 68 year-old man who was hospitalized for erythrodermic psoriasis refractory to multiple immunosuppressive and immunomodulatory therapies, ultimately developing sepsis due to bacteremia and fungemia complicated by infective endocarditis and a mycotic aneurysm. Although the widespread loss of epidermal function in erythroderma increases the risk of infection by opportunistic pathogens, water loss, and electrolyte imbalances, there are very few reported cases of psoriatic erythroderma complicated by fungemia and mycotic aneurysm. Given the high mortality associated with widespread epidermal dysfunction, there is a great need for evidence-based treatment guidelines for psoriatic erythroderma.

J Drugs Dermatol. 2024;23(8): doi:10.36849/JDD.7751

INTRODUCTION

Erythroderma is characterized by a widespread erythematous rash involving >90% of body surface area (BSA).1 It is frequently seen in the setting of pre-existing chronic inflammatory dermatoses such as psoriasis, atopic dermatitis, pityriasis rubra pilaris (PRP), and seborrheic dermatitis.2 The estimated prevalence among psoriasis patients is reported to be approximately 1% to 2.25%.1 Other causes include drug eruption, internal malignancy, erythrodermic mycosis fungoides, or Sezary syndrome. However, in a significant number of patients, the cause remains idiopathic despite extensive evaluation.2

Erythrodermic psoriasis (EP) is an uncommon and severe form of psoriasis with a high morbidity and increased mortality compared to other forms of psoriasis. Erythroderma typically requires hospitalization because of its possible complications, which include infection and sepsis, fluid and electrolyte loss, thermoregulatory disturbance, high-output cardiac failure, and respiratory distress.3 A 12-year-long prospective tertiary-care-center study of 309 erythroderma patients found that 9.1% of study participants died, most commonly due to sepsis and cardiovascular complications.4 A larger share of deaths were observed within the Sezary syndrome and mycosis fungoides group compared to other etiologies, which is a common cause of variation in the mortality rates found in other prognostic studies of erythroderma patients.5 Though there are several treatment options, limited evidence exists regarding the optimal treatment algorithm, which can make refractory cases challenging. Herein, we report a case of a 68-year-old patient with erythroderma secondary to severe, psoriasis, refractory to multiple first-line agents and complicated by septic shock, infective endocarditis, and a mycotic aneurysm.

Case Presentation
A 68-year-old man with no significant medical or dermatologic history was admitted for evaluation of a generalized rash. The patient reported that the rash began one year prior to presentation and was initially localized to the scalp with gradual cephalocaudal spread to the trunk and extremities. In addition to treatment with topical steroids (triamcinolone and betamethasone), mycophenolate was initiated, and a prednisone taper was completed, one week prior to presentation.
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