Pustular Psoriasis
Psoriasis is a polygenic, chronic inflammatory skin disease linked to dysregulation of the immune system that classically presents as sharply demarcated erythematous plaques with silvery scale [1, 2]. In some patients, the condition can manifest as a gene
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Pustular Psoriasis Sebastian Bernardo and Mark Lebwohl
42.1
Introduction
Psoriasis is a polygenic, chronic inflammatory skin disease linked to dysregulation of the immune system that classically presents as sharply demarcated erythematous plaques with silvery scale [1, 2]. In some patients, the condition can manifest as a generalized pustular variant that can follow a severe, potentially life-threatening course that demands urgent dermatological evaluation and treatment [1]. While pustules may be a part of the clinical picture in both pustular psoriasis and acne vulgaris, comedones are classically absent in the former. Further, pustular psoriasis commonly spares the face, even in its generalized form. Unlike severe acne, patients presenting with pustular psoriasis may be toxic appearing on physical exam and be found to have hematological abnormalities and electrolyte imbalances that represent a true dermatologic emergency.
42.2
Background
Psoriasis is the most prevalent autoimmune disease in the United States. Affecting approximately 2.2 % of the American population, psoriasis is associated with significant patient morbidity and is estimated to cost $11.25 billion in direct and indirect health care costs annually [3, 4]. S. Bernardo, M.D. Department of Dermatology, Mt. Sinai School of Medicine, 161 West 86th Street, Apt 1a, New York, NY 10024, USA M. Lebwohl, M.D. (*) Department of Dermatology, Mt. Sinai School of Medicine, 5 East 98th Street, 5th Floor, New York, NY 10029, USA e-mail: [email protected] J.A. Zeichner (ed.), Acneiform Eruptions in Dermatology: A Differential Diagnosis, DOI 10.1007/978-1-4614-8344-1_42, © Springer Science+Business Media New York 2014
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S. Bernardo and M. Lebwohl
Although pustular psoriasis is reported to occur in less than 5 % of individuals with psoriasis, its potential for severe complications and mortality warrants immediate workup and appropriate management [1, 2]. Pustular psoriasis can present in childhood but is more commonly seen in middle-aged adults [5, 6]. While adult men and women are affected equally, pustular psoriasis is more common in boys than girls by a ratio of 3:2 among pediatric patients [1, 6, 7]. When all psoriatic variants are included, Caucasians are more commonly affected than African Americans (2.5 % vs. 1.3 %) [8]. As an individual subtype, however, pustular psoriasis has no racial predilection [9, 10]. The pathophysiology underlying pustular psoriasis has not been clearly delineated. Cutaneous cytokine and immune dysregulation coupled with environmental triggers are most likely responsible. HLA-B27, HLA-Aw19, and HLA-Bw35 have all been described as haplotypes that increase the likelihood of a pustular psoriatic phenotype [2]. Genetic predispositions that contribute to alterations in the cytokine milieu of the skin may help to explain the complex tissue alterations associated with pustular psoriasis. Upregulation of proinflammmatory cytokines such as TNF-α, Il-8, VEGF, and growth-related oncogene (Gro)-alpha in conjunction with reduced activ
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