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Skin disease
Published in Catherine Nelson-Piercy, Handbook of Obstetric Medicine, 2020
Rarely, a severe form of generalized pustular psoriasis, impetigo herpetiformis, may develop. Urticated erythema, beginning in the flexures and especially the groins, is associated with sterile pustules, which may become widespread and affect mucosa. This condition is associated with severe systemic upset including fever, neutrophilia and hypocalcaemia. An increased risk of low-birthweight babies is reported and these women require intensive treatment with systemic corticosteroids and regular fetal surveillance. Impetigo herpetiformis often recurs in subsequent pregnancies.
Unexplained Fever Associated With Cutaneous Manifestations
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Among the main skin diseases in this group, an unexplained fever may be found in: Generalized pustular psoriasis (von Zumbush), a dramatic change in the nature of the common psoriasis, with an abrupt onset of high fever, increasing leukocytosis and severe malaise, accompanying the pinpoint pustules which develop on pre-existing psoriatic lesions.Impetigo herpetiformis, a rare minute pustular eruption starting on acutely inflammed areas of body skin, occurring especially in pregnant women, may be associated with a continuous or remittent fever, increasing with successive waves of the disease.
Dermatological emergencies in pregnancy
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Riti Bhatia, Neirita Hazarika, Ruby Bhatia
Impetigo herpetiformis or generalized pustular psoriasis of pregnancy is a rare, life-threatening pustular dermatosis related to pregnancy. Factors such as high progesterone during the last trimester of pregnancy, low calcium, and reduced epidermal skin–derived antileukoproteinase/elafin activity are implicated in formation of epidermal pustules.
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).
A review of disease burden and clinical management for generalized pustular psoriasis in China
Published in Expert Review of Clinical Immunology, 2022
For patients with early disease onset of pustular psoriasis and slow disease progression (slow disease progress: no marked changes in the standardized clinical outcome measures, such as GPPASI, GPPGA and BSA, are observed in patients, suggesting that no symptoms of the acute phase of the disease occur in patients with GPP), prognosis is promising [68]. Prognosis tends to be poor in: patients who experience rapid disease progression, particularly those with comorbid plaque psoriasis, with inadequate treatment response or those with extracutaneous complications who are receiving treatment. For elderly patients with uncontrolled GPP, complications (e.g. heart failure and respiratory tract infection) can affect prognosis. The prognosis for children with GPP can be promising if MTX or systemic hormones, such as prednisone, are avoided. Patients with subacute annular GPP or impetigo herpetiformis with a clear etiology generally have a good prognosis [69].
Generalized pustular psoriasis is a disease distinct from psoriasis vulgaris: evidence and expert opinion
Published in Expert Review of Clinical Immunology, 2022
Hervé Bachelez, Jonathan Barker, A. David Burden, Alexander A. Navarini, James G. Krueger
GPP has various forms with differing presentation and severity [2,15,28,47]. The most typical form is acute (or von Zumbusch) GPP, associated with widespread pustules and systemic effects. Population-specific subtypes also present: GPP of pregnancy (previously known as impetigo herpetiformis) and neonatal/infantile/juvenile GPP. Furthermore, acute forms can be further divided into GPP with or without concomitant PV [3,47]. As such, it is incorrect to consider GPP part of a continuum of PV severity or an acute form, rather than a condition with its own subtypes and manifestations.