Pustular psoriasis of pregnancy: current perspectives

Abstract

Pustular psoriasis of pregnancy (PPP) is a life-threatening condition for both the pregnant mother and fetus, and must be efficiently and accurately diagnosed and treated. This condition has historically been classified as a unique, separate dermatosis of pregnancy. However, current opinion and data suggest that it may be a variant of generalized pustular psoriasis. PPP typically occurs in the third trimester and is characterized by widespread coalescent pustules, desquamation, and systemic symptoms. Clinical features and histopathologic evaluation aid in diagnosis. Treatments during pregnancy include high-dose corticosteroids, cyclosporine, narrow-band ultraviolet B radiation, infliximab, granulocyte and monocyte adsorptive apheresis, and systemic antibiotics. Both the mother and fetus should be closely monitored with appropriate laboratory studies for the duration of the pregnancy and postpartum.

An update on generalized pustular psoriasis

“…Laboratory test findings in patients with GPP commonly include elevated C-reactive protein, leukocytosis and neutrophilia, and liver function abnormalities [4,10]. GPP may be classified into the following types: acute GPP (von Zumbusch variant; representing more than 50% to more than 90% of adult cases [3,11,12]), pustular psoriasis of pregnancy (previously referred to incorrectly as 'impetigo herpetiformis' [13]), and infantile/juvenile pustular psoriasis [14]. Pustular psoriasis may occur in localized forms, including palmoplantar pustular psoriasis and acrodermatitis continua of Hallopeau (ACH) [14].…”

“…In addition, acute generalized exanthematous pustulosis (AGEP) shares many features in common with GPP [16], and has been described as a drug-induced form of pustular psoriasis, although the clinical features, treatments, course, and triggers differ substantially from GPP [4]. The main clinical and histological features of GPP are summarized in Table 1 [4,13,14,[17][18][19][20] and Figure 1a [21]. Skin lesions typically observed in patients with GPP are shown in Figure 1b.…”

“…Oral corticosteroids may be considered in GPP if other treatment choices are not possible, but these agents should be used with caution and with gradual tapering of medication, as rapid withdrawal may induce a pustular flare. Psoralen ultraviolet (UV)-A phototherapy may be considered as second-line treatment in adults Anti-TNF-α agents Limited data available; greater number of reports of pregnancies exposed to infliximab than either adalimumab or etanercept [58] Infliximab [13,14] IV; 5 mg/kg at weeks 0, 2, & 6 [56] Rapid onset, effective for acute disease; limit use to first 30 weeks' gestation [13] B [14] Topical therapy UVB [14] Topical; 69 mJ/cm 2 3 times weekly increased at 10% increments with each treatment for sessions 1-34 and by 5% increments for treatments [35][36][37][38][39][40][41][42] Often effective when resistant to topical corticosteroids Safe [14] Corticosteroids [14,17] Topical; mild disease, standard dose Unknown Use lowest strength possible [14] *Lettering system per pre-2015 FDA pregnancy risk categories (revised pregnancy and lactation labeling has yet to be applied to all medications [13]); category B = animal reproduction studies failed to demonstrate risk to fetus and there are no adequate/well-controlled studies in pregnant women; category C = animal reproduction studies have shown an adverse effect on fetus and there are no adequate/well-controlled studies in pregnant women, but potential benefits may warrant use of drug in pregnant women despite potential risks; category D = positive evidence of human fetal risk based on adverse reaction data from investigational or marketing experience or studies in humans, but potential benefits may warrant use of drug in pregnant women despite potential risks.…”

“…Data for the use of systemic therapy for GPP in pregnant and lactating females are also limited. Treatment options include cyclosporine (at a reduced dose), oral corticosteroids, topical agents, and phototherapy with narrow-band UV-B [13]. Controlled clinical data are lacking on the safety of biologics during pregnancy in patients with psoriasis [58]; however, these agents are generally classified as FDA pregnancy risk category B (under the pre-2015 lettering scheme [86]) [13].…”

“…Treatment options include cyclosporine (at a reduced dose), oral corticosteroids, topical agents, and phototherapy with narrow-band UV-B [13]. Controlled clinical data are lacking on the safety of biologics during pregnancy in patients with psoriasis [58]; however, these agents are generally classified as FDA pregnancy risk category B (under the pre-2015 lettering scheme [86]) [13]. Currently, anti-TNF-α agents can be used during the first half of pregnancy (due to active transplacental transport after gestational week 22), and longer-term use may be considered on an individual patient basis depending upon disease severity [58].…”