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Erythrodermic and Pustular Psoriasis
Published in John Y. M. Koo, Ethan C. Levin, Argentina Leon, Jashin J. Wu, Alice B. Gottlieb, Moderate to Severe Psoriasis, 2014
Ethan C. Levin, Eric Y. Sako, Shannon Famenini, Jashin J. Wu
Pustular psoriasis has been historically classified into two forms: localized and generalized. Generalized pustular psoriasis can be further divided based on the acuity of presentation and natural history of disease. Annular pustular psoriasis usually follows a chronic, more benign course. In contrast, the von Zumbusch type is often a more severe presentation, characterized by the rapid onset of widespread sterile pustules on a background of erythema (Figure 17.2) [15]. Like erythrodermic psoriasis, preexisting psoriasis vulgaris may or may not be present [16]. Initially, pustules may be located at the periphery of psoriatic plaques, where inflammation is the most active. However, once the disease becomes generalized, it can develop on any involved skin [17]. Confluence of these pustules may form irregular “lakes of pus” over part or all of the body. The patient may appear toxic, with fever, chills, pain, and intense pruritus [18]. Other possible findings include a geographic or fissured tongue, scaly lips with superficial ulcerations, and ocular involvement (e.g., conjunctivitis, uveitis, or iritis). Histopathological examination is characterized Kogoj’s spongiform pustules, a subcorneal neutrophilic infiltrate with damaged or swollen keratinocytes as well as the classic histologic findings of psoriasis (i.e., parakeratosis, hyperkeratosis, elongated rete ridges, Munro’s microabscesses, and superficial perivascular lymphocytic infiltrate). The inflammation and edema are more severe than in the other subtypes of psoriasis [18].
Pustular psoriasis
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Santanu Banerjee, Neerja Saraswat
Topical corticosteroid therapy may be effective for the treatment of children with annular pustular psoriasis, which exhibits less severe manifestations than acute GPP. In addition, topical compresses, wet wraps, or oatmeal baths may be helpful for soothing the skin lesions. Topical therapy for annular pustular psoriasis is less practical when a large proportion of the body surface is affected. Patients who cannot be managed only with topical therapy can be treated with systemic agents.
Generalized pustular psoriasis: the new era of treatment with IL-36 receptor inhibitors
Published in Journal of Dermatological Treatment, 2022
Cláudia Oliveira Maçães, Ana Maria Lé, Tiago Torres
Generalized pustular psoriasis (GPP) is a rare but severe variant of psoriasis, characterized by eruption of superficial sterile pustules that appear suddenly and widely distributed in the body (1). Owing to its multisystemic affect, with extracutaneous organ involvement, it may be life-threatening. Acute GPP of von Zumbusch is the most common presentation, but others exist such as impetigo herpetiformis in association to pregnancy, and, less frequently, annular pustular psoriasis and juvenile pustular psoriasis (2,3). This type of psoriasis can appear with or without previous history of plaque psoriasis and most commonly presents in adults, although pediatric cases of GPP have been reported (1,4). It is predominantly reported in Asia, particularly in Japan, with a prevalence of 7.46 per million, while its prevalence in the Caucasian population is considerably lower (1.76 per million) (1).
Efficacy and safety of acitretin monotherapy in children with pustular psoriasis: results from 15 cases and a literature review
Published in Journal of Dermatological Treatment, 2018
Pingjiao Chen, Changxing Li, Rujun Xue, Huiheng Chen, Xin Tian, Kang Zeng, Xibao Zhang, Jingyao Liang
Pustular psoriasis (PP) is an uncommon heterogeneous form of psoriasis characterized by sterile pustules on normal-appearing or inflamed erythematous skin (1). It has been classified into generalized and localized forms. Depending on the clinical manifestations, the PP is divided to the following three subtypes such as: generalized pustular psoriasis (GPP, also known as von Zumbusch), annular pustular psoriasis (APP), and pustular erythrodermic psoriasis (PEP) and also the localized form is divided into two clinical groups: palmoplantar psoriasis (PPP) and acrodermatitis continua of Hallopeau (ACH) (1,2). PP has significant negative impact on quality of life and even is potentially life threatening in some severe cases. It affects approximately 1.1% of the childhood psoriasis in China (3) and has a worldwide prevalence of 0.6 to 7.0% in children with psoriasis (4,5).
Biologics in the treatment of pustular psoriasis
Published in Expert Opinion on Drug Safety, 2020
We performed a literature search of Pubmed published from inception to 31 May 2020. The following terms were used for the search: ‘biologics,’ ‘generalized pustular psoriasis,’ ‘acrodermatitis continua of Hallopeau,’ ‘palmoplantar pustular psoriasis,’ ‘palmoplantar pustulosis,’ and ‘pustular psoriasis.’ Clinical trials, open label studies, retrospective studies, case reports concerning the use of biologics in pustular psoriasis were included. The use of biologics in annular pustular psoriasis and impetigo herpetiformis were not included in this review.