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
There generalized pustular psoriasis (Gpp, from the English acronym Generalized pustular psoriasis) a serious rare skin disease, which manifests itself with arash 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. To bring patients together the lived marked by pain and lonelinessin addition to difficulty in obtaining a diagnosis quickly and the lack of care pathways dedicated to take charge. Learn about the pathology and the impact it has on the health and lives of people who suffer from it, but also solicit concrete regulatory proposals for taking charge of patientsthe goal of the campaign On my skin: living with GPPpresented to 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 adiffuse eruption of sterile pustules on an erythematous base which manifests itself 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 thenegative impact on quality of life of the patient.
The very extensive forms of the disease can cause potentially life-threatening complicationsfrom 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 -. Pathology has a And
impact on patients’ lives and its clinical progress is likely to go out of control if you don’t intervene promptly with correct therapies.
Still unrecognized rare
Generalized pustular psoriasis not yet recognized as a rare diseasei.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, although included in the portal of rare diseases, does not yet have an exemption code as a rare disease, so the taking charge remains relegated to chronic aspects only – explained Senator Mazzella, who presented a parliamentary question for the integration of GPP in the list of rare diseases -. It 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. We join thelengthening the time required 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 for reduce the impact not only on the clinical aspect but also in relational and working life.
Simplified routes
Patients need information and knowledge, of psychological support and to be able to rely on a trusting relationship with the attending physicianbecause the treatment path is long and not always the immediate and effective response to drugs – underlines Valeria Corazza, president of APIAFCO-Italian psoriatic association friends of the Corazza Foundation -. Are needed simplified routes 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, a lot can be done in rare diseases hard to find correct and up-to-date 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 extremely serious impact of this pathology on the life of those who suffer from it is understood, and therefore adequate procedures for taking charge and acknowledging rights.
The exhibition and the competition
The On My Skin: Living with GPP initiative takes its cue from Unwearable Collection, a collection of four statues created by the American artist Bart Hess, which visually represent four aspects of patients’ lives: the loneliness, pain, danger to life, burning experienced in the acute stages 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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