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Pustular psoriasis

Pustular psoriasis is a rare and severe inflammatory disorder, classified as a variant of , characterized by the abrupt onset of sterile, pus-filled pustules superimposed on areas of tender, erythematous . These pustules are typically 1–10 mm in diameter, contain neutrophils, and arise within hours to days, often leading to as they rupture and dry, forming lakes of that evolve into scaly plaques. Unlike common plaque , pustular psoriasis frequently involves systemic manifestations, including fever, , , and , making it potentially life-threatening if untreated. It affects approximately 1–2% of individuals with , with an estimated global of 1–7 cases per million persons, and can occur independently or in those with a history of other psoriasis forms. The condition encompasses several subtypes, including generalized pustular psoriasis (GPP), the most severe form often classified under the von Zumbusch subtype, featuring widespread involvement of the trunk, extremities, and flexural areas; and localized variants such as palmoplantar pustulosis on the palms and soles, acrodermatitis continua of Hallopeau starting at the fingertips or toes, and pregnancy-associated pustular psoriasis () arising in the third trimester. It exhibits a bimodal age distribution, affecting infants and adults aged 40–60 years, with no strong gender predominance, though it is more prevalent in individuals of Asian descent.

Overview

Definition

Pustular psoriasis is a rare, severe variant of characterized by the abrupt onset of sterile, pus-filled pustules on erythematous , without evidence of underlying . These pustules arise from immune-mediated and are filled with neutrophils rather than infectious agents, distinguishing the as a neutrophilic dermatosis. The core pathological features include dense neutrophilic infiltration within the , forming characteristic subcorneal collections known as Kogoj's spongiform pustules, alongside widespread and the potential for systemic inflammatory effects. This contrasts with the more common plaque , which primarily manifests as thickened, scaling plaques due to epidermal hyperproliferation without prominent pustule formation, and carries a lower risk of sudden, life-threatening flares. The classic generalized form, termed von Zumbusch pustular psoriasis, was first described in 1910 and exemplifies the acute, widespread presentation of the disease.

Epidemiology

Pustular psoriasis is a rare variant of , with generalized pustular psoriasis (GPP) exhibiting an incidence of approximately 0.06 to 1.5 cases per 100,000 person-years in various populations. Palmoplantar pustulosis (PPP), a localized subtype, demonstrates higher rates, with prevalence ranging from 1.1 to 18.7 per 100,000 persons in studies from the and . Overall, pustular forms account for 1-2% of all cases, underscoring their infrequency compared to plaque psoriasis. Demographically, GPP shows a bimodal age distribution, with peaks in onset during young adulthood (20-29 years) and (50-59 years), though cases occur across all ages, including infancy. A slight female predominance is observed in generalized forms, with female-to-male ratios ranging from 1.5:1 to 2:1, particularly in pregnancy-associated variants like impetigo herpetiformis, which exclusively affects pregnant women. This subtype often manifests in the third and resolves postpartum, highlighting a unique demographic link to . Globally, prevalence varies significantly, with higher rates reported in ; for instance, GPP prevalence reaches 7.46 per million in , compared to 1.5-45.2 per million in Western countries like and . These disparities are partly attributed to higher frequencies of IL36RN gene mutations in Asian populations, which predispose individuals to GPP. Underdiagnosis is likely in low-resource settings due to limited access to specialized dermatological care and challenges in distinguishing pustular variants from infections. Historically, severe untreated GPP carried mortality rates up to 15-24%, primarily from secondary infections and organ failure, but modern treatments have reduced this to 0-4.2% in hospitalized cases, reflecting improved supportive care and targeted therapies.

Pathophysiology

Genetic Factors

Pustular psoriasis is strongly associated with specific genetic that disrupt inflammatory signaling pathways, with loss-of-function in the IL36RN gene representing the most common and impactful genetic defect. The IL36RN gene encodes the interleukin-36 (IL-36Ra), which normally inhibits IL-36 signaling; lead to deficient IL-36Ra production, resulting in unchecked activation of IL-36 agonists (IL-36α, β, and γ). This dysregulation promotes excessive downstream signaling through and MAPK pathways, culminating in heightened expression of pro-inflammatory cytokines and that drive neutrophilic and pustule formation. These are particularly prevalent in generalized pustular psoriasis (GPP), accounting for up to 80% of cases in certain populations, and are linked to the clinical known as deficiency of interleukin-36 (DITRA). Inheritance of IL36RN-related pustular psoriasis follows an autosomal recessive pattern, requiring biallelic mutations (homozygous or compound heterozygous) for full disease expression, though monoallelic variants may contribute to incomplete or milder phenotypes. Sporadic cases predominate, but familial clustering has been observed, underscoring the oligogenic nature of in some instances. Other genes implicated include CARD14, where gain-of-function mutations enhance activation and inflammatory gene transcription (e.g., CCL20 and IL8), primarily in GPP cases comorbid with vulgaris; these mutations are less common in isolated GPP and exhibit autosomal dominant . Additionally, loss-of-function variants in AP1S3, which impair adaptor protein complex-1-mediated vesicular trafficking and , have been associated with palmoplantar pustulosis, a subtype of pustular psoriasis, by upregulating IL-36 expression and activity; these are rarer in generalized forms. Rarer mutations, such as loss-of-function variants in MPO (), have also been implicated in a subset of cases, particularly in populations. Ethnic variations significantly influence mutation prevalence, with IL36RN defects showing higher rates in East Asian cohorts; for example, 46-82% of Japanese patients with GPP (particularly GPP alone, without vulgaris) harbor these , compared to 20-40% in European populations. Common IL36RN variants in East Asians include c.115+6T>C and c.28C>T, while Europeans more frequently carry c.338C>T. CARD14 , such as c.526G>C, are also enriched in Asian populations with GPP- vulgaris overlap, whereas AP1S3 variants predominate in Europeans with palmoplantar involvement. Genetic testing for IL36RN, CARD14, and AP1S3 plays a crucial role in confirming , especially in presentations, early-onset cases, or familial clusters, enabling targeted therapies like IL-36 inhibitors (e.g., spesolimab) that address the underlying molecular defect. Such testing helps differentiate pustular psoriasis from mimics and informs , as IL36RN carriers often experience more severe, recurrent flares with systemic involvement.

Triggers and Risk Factors

Pustular psoriasis flares are often precipitated by environmental, pharmacological, and physiological factors that disrupt immune in susceptible individuals. The of systemic corticosteroids is one of the most frequently reported triggers, often leading to severe rebound exacerbations that require hospitalization. Up to 50% of GPP flares overall require hospitalization. Infections, particularly streptococcal upper infections, are common precipitants that can initiate or worsen generalized pustular psoriasis episodes. , especially during the third trimester, is associated with the onset of herpetiformis, a variant of generalized pustular psoriasis linked to hormonal changes. Certain medications pose significant pharmacological risks for inducing pustular flares. Lithium and TNF-alpha inhibitors, such as and —often used to treat plaque —can paradoxically trigger pustulation in approximately 2-5% of exposed patients. , arising from conditions like or iatrogenic causes, has been documented to precipitate acute generalized pustular psoriasis, with resolution observed upon calcium correction. Other exacerbating factors include , which aggravates flares in annular and generalized forms, and alcohol consumption, which shows a strong association with pustular lesions due to its immunomodulatory effects. serves as a modifiable , correlating with increased flare severity and frequency in comorbid patients. A prior history of plaque substantially heightens susceptibility to pustular variants, affecting 31-78% of individuals who develop generalized pustular psoriasis. sex hormones contribute to risk in pregnancy-associated cases, where elevated progesterone levels may destabilize lesional . To mitigate these risks, gradual tapering of systemic corticosteroids is essential, as abrupt discontinuation heightens probability, while prompt management of and can prevent escalation.

Clinical Features

Signs and Symptoms

Pustular psoriasis manifests primarily through acute eruptions characterized by the sudden appearance of numerous small sterile pustules filled with white or yellow , superimposed on an erythematous base. In generalized forms, these pustules often coalesce into sheet-like distributions across extensive body areas, followed by rapid within 1-2 days that forms lakes of and subsequent . Patients commonly report associated sensations of pruritus, , or pain in the affected . Systemic symptoms frequently accompany active flares, including fever exceeding 38°C (reported in 24-96% of cases), chills, , , fatigue, and . Severe episodes may also involve , , and marked , with counts elevated up to 40,000/µL alongside . In localized variants such as palmoplantar pustulosis, the condition presents with recurrent crops of painful pustules on the palms and soles, often accompanied by , fissuring, and that impair daily function. involvement is common in these cases, featuring pitting, , subungual , and potential nail shedding. Acute flares generally last 7-14 days, though they can extend to 2-5 weeks in some instances, with pustules drying into scales or scabs. Pediatric presentations of pustular psoriasis are rare and often take an annular form, with circinate erythematous plaques featuring peripheral pustules and scaling, typically on the trunk, and may be triggered by infections such as those caused by . These variants tend to be milder and subacute compared to adult-onset generalized disease.

Subtypes

Pustular psoriasis encompasses several distinct clinical variants, primarily classified into generalized and localized forms based on the extent of skin involvement and associated systemic features. These subtypes differ in onset, distribution, and potential triggers, though they share histopathological features such as sterile neutrophilic pustules. Generalized pustular psoriasis (GPP), also known as von Zumbusch type, represents the most severe form, characterized by widespread eruption of sterile pustules, often accompanied by acute flares and systemic symptoms like fever and . It typically presents with rapid onset of diffuse followed by pinpoint pustules that coalesce into lakes, leading to ; variants include the classic von Zumbusch subtype (affecting approximately 90% of GPP cases with life-threatening potential), annular type (featuring circinate lesions with pustules at the periphery, developing over 7 days to 3 months), and inverse variant (pustules predominantly in flexural areas). Genetic mutations, such as in IL36RN, are implicated in a subset of GPP cases, particularly the von Zumbusch form. Palmoplantar pustulosis () is a , localized subtype confined to the palms and soles, featuring recurrent crops of yellow-brown sterile pustules on an erythematous base, often evolving into hyperkeratotic plaques with fissuring and scaling. It affects women more frequently and is strongly associated with , with a of 0.05–0.12%; unlike GPP, it rarely involves systemic symptoms but can cause significant and functional impairment. Acrodermatitis continua of Hallopeau () is a rare, progressive localized variant primarily affecting the distal extremities, beginning with pustules on the fingers and toes that lead to severe nail dystrophy, , and potential . It may extend proximally and is associated with sterile pustular inflammation; chronic cases can result in or in the phalanges, contributing to . ACH often occurs independently or between flares of GPP. Impetigo herpetiformis is a pregnancy-specific variant of GPP, typically emerging in the third trimester with annular or arrangements of pustules on erythematous skin, often in flexural regions, and resolving postpartum. It is frequently accompanied by and systemic features such as , posing risks to maternal and fetal health, including preterm delivery. Drug-induced pustular reactions mimicking the Zumbusch subtype, such as those from s or antibiotics, are not considered true pustular psoriasis subtypes but rather (AGEP), distinguished by shorter duration and different histopathology.

Diagnosis

Clinical Evaluation

The clinical evaluation of suspected pustular psoriasis begins with a thorough history taking to identify the onset, potential triggers, and associated features. Onset is typically acute, with pustules appearing rapidly over hours to days, often in patients with a background of plaque or prior episodes. Common triggers include systemic withdrawal, infections such as streptococcal or staphylococcal, , , or medications like and beta-blockers. Family history should be explored for or related genetic predispositions, such as variants in the IL36RN gene, while associated symptoms like fever, chills, arthralgias, fatigue, or pruritus help gauge systemic involvement. Physical examination focuses on the skin, nails, and systemic signs to assess the distribution and extent of lesions. Erythematous plaques studded with pinpoint sterile pustules are characteristic, often coalescing into lakes of pus on the trunk, extremities, and flexures, with rare involvement of the face or mucous membranes. The extent of erythema and pustules is evaluated using adaptations of the Psoriasis Area and Severity Index (PASI), such as the Generalized Pustular Psoriasis Area and Severity Index (GPPASI), which scores pustule density, erythema, and scaling across body regions while accounting for body surface area (BSA) involvement. Severity can be further assessed using the Generalized Pustular Psoriasis Physician Global Assessment (GPPGA), a 5-point scale from clear (0) to severe (4) based on pustules, erythema, and scaling. Vital signs, including temperature and hydration status, are checked for evidence of systemic illness, and nails are inspected for onycholysis or subungual pustules. For generalized pustular psoriasis (GPP), the 2024 International Psoriasis Council consensus defines it as a systemic inflammatory characterized by cutaneous and macroscopically visible sterile pustules. Essential diagnostic criteria include primary sterile pustules on non-acral erythematous , with supporting criteria such as fever (>38°C), elevated or other markers of , neutrophilic , and/or lakes of pus. For GPP flares, particularly the von Zumbusch subtype, features include widespread sterile pustules on erythematous accompanied by fever, , and , often requiring hospitalization. Red flags during evaluation include signs of secondary bacterial infection, such as non-sterile purulent discharge, , or worsening fever, as well as evidenced by , dry mucous membranes, or , which necessitate urgent medical . is integral to the evaluation, emphasizing recognition of early signs like new pustules or increasing to prompt timely , alongside avoidance of known triggers such as abrupt tapering.

Laboratory and Histological Findings

Skin biopsy is a key diagnostic tool for confirming pustular psoriasis, particularly when clinical features are ambiguous. The hallmark histological finding is Kogoj's spongiform pustule, characterized by collections of neutrophils within the , often accompanied by microabscesses. Other typical features include parakeratosis, acanthosis, and elongation of rete ridges, with Gram staining typically negative for organisms, confirming the sterile nature of the pustules. Laboratory evaluations support the diagnosis by revealing . Common findings include with , elevated (ESR), and increased (CRP) levels, reflecting the acute inflammatory response. In pregnancy-associated forms, such as impetigo herpetiformis, calcium may be normal or low, while cultures of pustular fluid are negative for infectious agents. Imaging studies are not routinely employed but may be indicated in specific subtypes. For acrodermatitis continua of Hallopeau (ACH), X-rays can occasionally reveal osteitis or bone resorption in affected digits, aiding in assessment of complications. Genetic testing, such as sequencing of the IL36RN gene, is reserved for atypical or early-onset cases to identify mutations associated with deficient IL-36 receptor antagonist production, though it is not a first-line diagnostic approach. Biopsy findings are particularly useful in the differential diagnosis, distinguishing pustular psoriasis from infectious conditions like bacterial folliculitis (where organisms are present on staining) or neutrophilic dermatoses such as Sweet syndrome (which lacks spongiform pustules and shows primarily dermal involvement).

Treatment

Pharmacological Therapies

Pharmacological therapies for pustular psoriasis primarily target and immune dysregulation, with treatment selection depending on the subtype—generalized pustular psoriasis (GPP), palmoplantar pustulosis (), or acrodermatitis continua of Hallopeau ()—and disease severity. For acute GPP flares, first-line options include cyclosporine at 3-5 mg/kg/day orally, which offers rapid onset of action within days due to its inhibition of T-cell activation, though long-term use is limited by risks of and . , a systemic , serves as another first-line agent for GPP flares and maintenance at 0.5-1 mg/kg/day orally, promoting epidermal differentiation and reducing hyperproliferation, with efficacy demonstrated in rapid clinical improvement but contraindicated in due to teratogenicity. Biologic therapies have revolutionized management, particularly for GPP. Spesolimab, an interleukin-36 receptor (IL-36R) inhibitor, is FDA-approved since 2022 for adult GPP flares (intravenous), with the subcutaneous formulation approved in 2024 for adults and pediatric patients aged 12 years and older weighing at least 40 kg to prevent flares, administered intravenously at 900 mg (with an optional second dose after one week) for acute use or subcutaneously at 600 mg loading followed by 300 mg every four weeks for maintenance; clinical trials showed a 54% response rate (GPPGA pustulation subscore of 0) at one week versus 6% with , and an 84% reduction in flares over 48 weeks. Off-label use of IL-17 inhibitors, such as (300 mg subcutaneously every four weeks) and ixekizumab (160 mg loading then 80 mg every four weeks), has shown efficacy in GPP and through blockade of IL-17A-driven inflammation, with case series reporting clearance of pustules and plaques. , an IL-12/23 inhibitor (45-90 mg subcutaneously at weeks 0 and 4, then every 12 weeks), is reserved for refractory cases, offering sustained remission in limited studies. Other systemic agents include at 15-25 mg/week orally or subcutaneously, which inhibits to suppress immune responses and is suitable for maintenance in GPP or , though monitoring for is required. Systemic corticosteroids are generally avoided due to the high risk of rebound flares upon withdrawal, as evidenced by real-world data showing frequent prescriptions despite this complication. For localized forms like PPP and ACH, topical therapies predominate, including potent corticosteroids (e.g., clobetasol 0.05% ointment twice daily) combined with calcipotriol (50 mcg/g ointment twice daily) to reduce pustule formation and scaling, often under occlusion for enhanced penetration. Phototherapy, such as psoralen plus UVA (PUVA) administered 2-3 times weekly, serves as an adjunctive option for PPP and ACH, yielding comparable efficacy to systemic agents in select patients when combined with retinoids. Emerging therapies as of 2025 target the IL-36 pathway beyond spesolimab, with additional agents in development for GPP flares. Vunakizumab, an IL-17A (240 mg subcutaneously at weeks 0, 2, 4, then every four weeks), shows promise in a for , achieving pain resolution within one week and significant improvement by 10 weeks in , though larger studies are ongoing to confirm in pustular variants.

Supportive Measures

Supportive measures for pustular psoriasis focus on alleviating symptoms, maintaining physiological balance, and preventing complications during acute flares, particularly in generalized forms where systemic involvement is prominent. In severe cases of generalized pustular psoriasis (GPP), hospitalization is often necessary to provide comprehensive care, including to minimize energy expenditure and prevent excessive heat loss from widespread involvement. Hydration is a critical component, as patients may experience significant fluid loss due to fever, poor oral intake, and insensible losses from denuded skin; intravenous fluids are administered to correct and maintain balance. In instances associated with , which can occur due to calcium in pustules or underlying parathyroid issues, calcium levels should be monitored closely, with supplementation provided if deficiency contributes to the flare. Bland topical compresses and saline or baths are recommended to soothe discomfort, promote gentle of pustules, and support skin barrier recovery without causing further irritation. Skin care emphasizes gentle, non-irritating interventions to prevent fissuring and secondary damage to fragile, pustule-covered skin. Emollients are applied liberally to hydrate and protect the skin barrier, while avoiding harsh soaps, hot water, or abrasive fabrics that could exacerbate or pustule rupture. For localized forms such as palmoplantar pustulosis (), wet wrap therapy— involving damp layers over emollients—can enhance moisture retention and reduce in affected areas like the palms and soles. Aseptic techniques are essential during dressing changes to minimize the risk of bacterial superinfection, with antibiotics reserved for confirmed secondary infections, such as those caused by , rather than prophylactic use. Nutritional support may be required in hospitalized patients with prolonged flares leading to hypercatabolism or inadequate intake, ensuring caloric and protein needs are met to aid recovery. modifications play a preventive role; for , is particularly beneficial, as use is a known trigger and quitting has been shown to improve severity and reduce risk. techniques, such as relaxation exercises, are advised across subtypes to mitigate flare induction, given the bidirectional link between and exacerbations. Patients should be educated on recognizing early warning signs, like new pustules or fever, to seek prompt care and avoid known triggers such as .

Prognosis

Disease Course

Pustular psoriasis typically follows a relapsing-remitting course, with episodes of acute flares interspersed by periods of remission lasting months to years. In approximately 50% of cases, it transforms from preexisting , often triggered by factors such as withdrawal or infections. The acute phase manifests with rapid onset over hours to days, featuring widespread sterile pustules on erythematous skin, accompanied by systemic symptoms like fever and , peaking at 48-72 hours and resolving within 1-2 weeks under treatment; occurs rarely without intervention. Flares are unpredictable in frequency and severity, with 73% of patients experiencing 1-2 episodes and 15% having more than four over several years. Several factors influence the disease trajectory, including early therapeutic intervention, which shortens flare duration and reduces hospitalization rates. Genetic mutations, such as in IL36RN or CARD14, are associated with earlier onset and more severe, recurrent disease. In pregnancy-associated forms (impetigo herpetiformis), symptoms often remit postpartum. Triggers like , , or medication changes precipitate relapses in over 50% of cases. Monitoring involves regular dermatologic follow-up to detect subclinical progression, using tools like the Generalized Pustular Psoriasis Area and Severity Index (GPPASI) and laboratory markers such as (CRP) and (ESR). Biologic therapies can achieve sustained remission in responsive patients, though many require ongoing treatment to prevent flares. Historically, prior to biologics, mortality from untreated or poorly managed pustular psoriasis ranged from 4% to %, primarily due to or multi-organ failure; contemporary supportive care and targeted therapies have significantly reduced this, with recent studies (as of 2025) reporting approximately 1% at 1 year and up to 6% long-term.

Complications

Pustular psoriasis can lead to several acute complications, particularly during severe flares of generalized pustular psoriasis (GPP). Secondary bacterial infections may rarely progress to , with an estimated in-hospital mortality rate associated with such events around 4.2%. Widespread can induce high-output cardiac failure due to increased metabolic demands and fluid shifts. In severe cases, (ARDS) may develop, characterized by noninfectious pulmonary inflammation and reported in up to 15 documented instances linked to GPP or psoriatic erythroderma. Chronic complications often involve persistent tissue damage and associated conditions. In acrodermatitis continua of Hallopeau (), a localized form of pustular psoriasis, progressive nail involvement can result in permanent or , leading to functional impairment and . Up to 50% of cases may overlap with , manifesting as joint inflammation and . Long-term use of retinoids, such as , for management carries a of hepatic , including elevated aminotransferase levels and acute in about 1.5% of cases. In pregnancy, impetigo herpetiformis—a variant of pustular psoriasis—poses significant risks if untreated, including that can cause and preterm delivery. Fetal outcomes may include or early neonatal death due to these complications. Treatment-related complications include rebound flares upon withdrawal of systemic corticosteroids, which can exacerbate pustulation and generalize the disease. Paradoxical pustulosis induced by anti-TNF biologics occurs in roughly 1-3% of treated patients, presenting as new-onset pustular lesions despite initial therapeutic intent. The visible disfigurement from widespread pustules and recurrent flares significantly impairs , contributing to psychosocial burdens such as low , , and emotional distress in affected individuals.