Pustular psoriasis

Pustular psoriasis (psoriasis pustulosa) is a rare, severe form of inflammatory psoriasis, characterized by the occurrence of sterile pustules on an erythematous base. This condition has an immunological basis and is associated with abnormal activation of the immune system, leading to excessive proliferation of keratinocytes and an intensified inflammatory reaction in the skin. Unlike classic plaque psoriasis, pustular psoriasis can progress rapidly, often with systemic symptoms such as fever or malaise. Generalized and localized forms (e.g., palmoplantar) are distinguished, which differ in clinical course and prognosis.

Pustular psoriasis – causes

The pathogenesis of pustular psoriasis is complex and involves the interaction of genetic, immunological, and environmental factors. A key role is played by the deregulation of the immune response, particularly within the axis of interleukins such as IL-36, IL-17, and TNF-α.

The most important etiological factors include:

Genetic predisposition – mutations in the IL36RN gene (encoding the interleukin-36 receptor antagonist) are particularly significant in generalized forms,
Sudden withdrawal of systemic glucocorticosteroids, which can trigger a rapid flare-up of the disease,
Bacterial and viral infections, especially streptococcal,
Psychological stress – as a factor modulating the immune response,
Pregnancy – in the case of so-called pustular psoriasis of pregnancy (impetigo herpetiformis),
Drugs – including lithium, beta-blockers, some antimalarial drugs,
Metabolic disorders, including hypocalcemia.

The mechanism of the disease is based on excessive activation of neutrophils, which accumulate in the epidermis, forming characteristic sterile pustules. This process occurs without bacterial infection, which distinguishes psoriatic lesions from purulent lesions of infectious etiology.

Pustular psoriasis – symptoms

The clinical presentation of pustular psoriasis depends on its form; however, the common denominator is superficial pustules filled with purulent content, forming on an erythematous, inflammatory base.

Typical skin symptoms:

small, sterile pustules, which may coalesce into larger foci,
redness and swelling of the skin,
desquamation of the epidermis after the resolution of pustules,
burning sensation and tenderness,
excessive dryness and cracking of the skin (especially within the palms and soles).

Systemic symptoms (especially in the generalized form):

fever,
chills,
weakness,
leukocytosis,
elevated ESR and CRP,
electrolyte disturbances.

In the palmoplantar form, lesions are limited to the surfaces of the palms and soles, often leading to significant functional impairment of the patient – pain and skin cracks make walking and performing daily activities difficult.

The course of the disease is recurrent, with periods of exacerbation and remission. In severe cases, pustular psoriasis can be a life-threatening condition, especially in the generalized form (von Zumbusch).

Pustular psoriasis – treatment

Treatment of pustular psoriasis requires a multidisciplinary approach and adjustment of therapy to the type and severity of the disease. Due to the potentially severe course, hospitalization is necessary in many cases.

Systemic treatment:

Retinoids (e.g., acitretin) – regulate the process of keratinization and differentiation of keratinocytes,
Methotrexate – has an immunosuppressive effect, inhibiting cell proliferation,
Cyclosporine – fast anti-inflammatory effect through inhibition of T lymphocytes,
Biological drugs:
- TNF-α inhibitors (e.g., adalimumab),
- IL-17 inhibitors (secukinumab),
- IL-23 inhibitors,
- modern therapies targeting IL-36 (especially in generalized forms).

Topical treatment:

high-potency glucocorticoids,
vitamin D3 analogs (calcipotriol),
keratolytic preparations (e.g., salicylic acid),
emollients restoring the epidermal barrier.

Phototherapy:

PUVA (psoralen + UVA),
UVB 311 nm – especially in localized forms.

Supportive dermatological and aesthetic medicine procedures

In remission phases and as a supplement to pharmacological therapy, procedures supporting skin regeneration and reduction of inflammation are used:

LED light therapies – anti-inflammatory and regenerative effects,
moisturizing treatments and those restoring the hydrolipid barrier,
medical peels with controlled keratolytic action,
skin mesotherapy – improvement of tissue trophicity and hydration.

In clinical practice, also as part of the procedures used at Ambasada Urody Clinic & SPA, treatments supporting skin condition are used, such as intensive skin regeneration, anti-inflammatory therapies, or treatments restoring the skin microbiome. Their goal is not causal treatment, but improvement of skin quality and prolongation of remission periods.

General management also includes:

elimination of triggering factors (e.g., stress, medications),
treatment of comorbidities,
appropriate skin care (dermocosmetics, emollients),
psychological support.