Dermatological conditions
David M. Luesley, Mark D. Kilby in Obstetrics & Gynaecology, 2016
Acne, erythema multiforme, erythema nodosum and generalised pustular psoriasis form the first group. Pre-existing acne may deteriorate during pregnancy, but may also present anew. Erythema multiforme and erythema nodosum are both caused by a multitude of other aetiological factors, which must be excluded before it is possible to attribute the onset to pregnancy alone. Generalised pustular psoriasis describes a superficial sterile eruption occurring on the background of widespread erythema, which is associated with fever, systemic upset and hypocalcaemia (with tetany) in the more severe cases. It carries significant perinatal mortality and is more common in those with a history of plaque psoriasis. A clinically identical condition called impetigo herpetiformis was previously thought to be a pregnancy-specific dermatosis, but the two are now considered the same condition. Pregnancy appears to be one of a number of triggers for generalised pustular psoriasis.
Rashes in Pregnancy (See also Itching in Pregnancy and Vulval Itching)
Tony Hollingworth in Differential Diagnosis in Obstetrics and Gynaecology: An A-Z, 2015
Impetigo herpetiformis looks similar to pustular psoriasis, except it is directly related to the pregnancy. It is the most dangerous of the pregnancy-related rashes and carries a small risk of maternal mortality. Clinically, skin lesions are tender, scaly, and red with a broad sheet or surrounding ring of studded pustules (Figs 4 and 5). The lesions may be widespread, but frequently affect flexural (especially the groin) and acral (hands and feet) areas. The patient can be quite unwell with pyrexia, a mild inflammatory hepatitis and general malaise. Treatment is often difficult. Systemic steroids are frequently necessary and, occasionally, other second-line, potentially more toxic preparations (e.g. methotrexate) are required. The fetus may be affected. Once the child is delivered, the rash usually settles. Fortunately, the condition is rare, but it can recur in subsequent pregnancies.
Unexplained Fever Associated With Cutaneous Manifestations
Benedict Isaac, Serge Kernbaum, Michael Burke in 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.
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.