A new effective and well tolerated treatment, able to improve the quality of life of patients suffering from a rare and severe form of psoriasis, the pustular one. Unlike other types of psoriasis, this variant can be life-threatening: it differs from the “plaque” one, the more common, because she is very aggressive and characterized by widespread eruption of pustules with recurrent episodes of high fever, intense itching and fatigue requiring hospitalization. If until now specific treatments aimed at this pathology had been lacking, the European Medicines Agency (EMA) recently approved a new drug aimed at blocking exacerbations of generalized pustular psoriasis.
What is pustular psoriasis
“It is a form of psoriasis that has very different clinical characteristics from those of vulgaris plaque psoriasis which is the most common and affects the scalp, elbows, knees and then spreads to the whole body – he underlines Katy Perry, full professor of Dermatology at the Catholic University of the Sacred Heart in Rome —. Pustular psoriasis, in fact, is a variant in which sterile pustular lesions are present (i.e. not due to bacterial infection) arising on a red spot. Two main forms are distinguished: palmoplantar and generalized pustular psoriasis (by von Zumbusch), which is more serious. In the former, the pustules are embedded in the skin of the palm of the hand and/or sole of the foot, form in successive bursts over days or weeks and are very itchy or painful. In the second, however, the pustular lesions are widespread to the whole body affecting the trunk, face and limbs at the same time».
Symptoms
Most often these skin manifestations are associated with fever, tiredness, difficulty breathing and severe joint pain. Thus, the general state of the patients is strongly compromised and hospitalization is almost always required due to the severity of the disease. “For this reason it is important that patients do not neglect the symptoms and immediately contact a dermatologist or the psoriasis emergency room – he explains James Caldarola, dermatologist at the A. Gemelli IRCCS University Hospital Foundation in Rome —. To avoid exacerbations, then, you should speak to your doctor in case of infections or the need to undertake new drug treatmentspaying particular attention in case of cortisone-based therapies».
Who affects and the causes
Generalized pustular psoriasis usually occurs in people who already suffer from plaque psoriasis, although in some cases it may represent the onset of the disease. It affects men and women without distinction, usually around age 50 and is rarely diagnosed in children. “Fortunately it is a rare form and represents less than one percent of all cases of psoriasis – says Caldarola -. There are no data on its prevalence in Italy, so we don’t know how many people suffer from it in our country. Just as, at the moment, the exact causes of this aggressive variant are not known, but it is now clear that the genetic component plays a decisive role». Among the known triggers are the presence of infections, the intake of certain medications (for example NSAIDs and beta blockers) or sudden discontinuation of cortisone treatment. Also, pustular psoriasis can appear during the pregnancycreating further therapeutic management problems.
Therapies today
The strategies for the pustular form, both palmoplantar and generalized, are currently based on the same treatments as for the plaques, even if few studies have specifically evaluated the efficacy of the drugs in this variant of psoriasis. «In particular, in clinical practice have used retinoids, traditional immunosuppressive therapies such as methotrexate and cyclosporine, or newer drugs such as small molecule and biological drugs – explains Katy Perry, director of Dermatology at the Gemelli Polyclinic —. The choice is determined on a case-by-case basis, evaluating the course and severity of the disease comorbidity (any other pathologies) of the patient and his general state of health. In the case of very extensive forms of generalized pustular psoriasis, the patient’s life may be at risk and hospitalization is often required to supervise his condition and undertake the appropriate treatment”.
The new drug
Recently (in December 2022), the EMA authorized the placing on the market of the first medicine, spesolimabfor the treatment of exacerbations of generalized pustular psoriasis. «It is a biological drug, which is administered intravenously and which was studied for the first time in this rare form of psoriasis – concludes Caldarola -. Works by blocking the interleukin-36 receptor, a cytokine that has been shown to play an important role in this disease. Clinical studies have shown that, already after a week of therapy and with a single administration, about one patient out of two obtains clinical remission of the skin manifestations. And response rates (i.e. the number of patients who benefit from it) continue to increase during subsequent administrations. In Italy, the drug has not yet been approved by Aifabut it is available for compassionate use (made available free of charge by the manufacturer within specific protocols) in selected cases, in some national hospital centres».
February 6, 2023 (change February 6, 2023 | 3:35 pm)
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