by Health Editor
Although the pathology is extremely rare, it has not yet been included in the list of rare diseases (Annex to the essential levels of assistance). It means not being able to make use of the support of centers specialized in early diagnosis and treatment
Generalized pustular psoriasis (GPP) is a serious rare skin disease, which manifests itself with a rash of yellow-brown pustules of various sizes, often accompanied by pain, burning and itching. It is estimated that in Italy about 150 people in Italy suffer from it. What the patients have in common is the experience marked by pain and loneliness, as well as the difficulty of having a diagnosis quickly and the lack of dedicated care pathways for taking charge. Raising awareness of the pathology and the impact it has on the health and lives of the people who suffer from it, but also soliciting concrete regulatory proposals for taking care of patients, the objective of the campaign On my skin: living with GPP, presented in the Senate during a meeting organized by Senator Orfeo Mazzella, president of the Parliamentary Intergroup for Rare and Oncological Diseases.
The disease, diagnosis, symptoms
Generalized pustular psoriasis is a rare, life-threatening, autoinflammatory skin disease characterized by a widespread eruption of sterile pustules over an erythematous base manifesting with or without systemic symptoms such as pain, fever, general malaise, fatigue, and extracutaneous manifestations (cholestasis, arthritis ) – explains Maria Concetta Fargnoli, vice president of SIDeMaST – Italian Society of Dermatology, Venereology and Sexually Transmitted Diseases – . The clinical course of GPP is highly variable; in general, without treatment, unstable and long-lasting. The important cutaneous involvement, the associated symptoms (itching and pain), the systemic symptoms and the comorbidities contribute to the negative impact on the patient’s quality of life.
The very extensive forms of the disease can cause potentially life-threatening complications, from sepsis to necrosis of the renal tubules, from liver damage to cardiorespiratory failure.
Early diagnosis is fundamental – underlines Francesco Cusano, president of ADOI – Association of Italian hospital dermatologists-venereologists and public health -. The pathology has a high impact on the life of patients and its clinical trend risks going out of control if timely intervention is not taken with correct therapies.
Still unrecognized rare
Generalized pustular psoriasis not yet recognized as a rare disease, i.e. not included in the list of rare diseases (Annex 7 to the DPCM updating the Lea, the essential levels of assistance) but in that of chronic diseases. The disease, despite being included in the rare disease portal, does not yet have an exemption code as a rare disease, so taking charge remains relegated to chronic aspects only – explained Senator Mazzella, who presented a parliamentary question to the inclusion of GPP in the list of rare diseases –. He means that, if a diagnostic doubt arises, patients are forced to bear all the costs for any diagnosis, not being able to make use of the support of specialized centers in the area. This is associated with the lengthening of the time necessary to arrive at a diagnosis which, inevitably, exposes people to a high risk for their own lives. It is a rare but also chronic disease – adds Paola Pisanti, expert on rare diseases and consultant to the Ministry of Health -. In the light of the PNRR and the DM 77, it will be necessary to implement an effective coordination of all the actors involved in the management of the GPP: Community hospitals, Community homes, specialist centres, general practitioners and pediatricians of free choice and the Community to reduce the impact not only on the clinical aspect but also on relational and working life.
Simplified routes
Patients need information and knowledge, psychological support and to be able to count on a relationship of trust with the treating doctor, because the treatment path is long and the response to drugs is not always immediate and effective – underlines Valeria Corazza, president of APIAFCO – Italian psoriatic association friends of the Corazza Foundation -. Simplified pathways are needed, avoiding long waiting lists and getting to the specialist promptly.
The information campaign
The campaign On my skin: living with GPP promoted by Boehringer Ingelheim Italy, ADOI-Association of Italian Hospital Dermatologists-Venereologists and Public Health, SIDeMaST-Italian Society of Dermatology, Venereology and Sexually Transmitted Diseases, APIAFCO- Italian Psoriatic Association Friends of the Foundation Corazza, OMaR-Observatory for Rare Diseases. For the first time, an alliance of scientific societies and associations is working towards a common commitment for patients living with GPP. In general, says Ilaria Ciancaleoni Bartoli, director of OMaR, in rare diseases it can be very difficult to find correct and updated information. This also applies to GPP, where diagnosis is still an issue. It is also necessary to raise awareness at all levels so that the very serious impact of this pathology on the life of those who suffer from it is understood, and therefore suitable paths for taking charge and recognizing rights are organised.
The exhibition and the competition
The initiative On my skin: living with GPP takes its cue from the Unwearable Collection, a collection of four statues created by the American artist Bart Hess, which visually represent four aspects of patients’ lives: loneliness, pain, danger to life , the burning that is felt in the acute phases of the disease. The students of the Milan Academy of Luxury and Fashion will also be involved in the awareness campaign, who will design garments that represent the patients’ experiences and the need for solutions. The works, selected by a jury of experts, will be presented during Milan Fashion Week in September, when the statues of the Unwearable Collection will also be exhibited for the first time in Italy.
June 27, 2023 (change June 27, 2023 | 19:52)
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