The pathology will be discussed at the 61st National Congress of the Association of Italian Hospital Dermatologists-Venereologists, which will take place from 10 to 12 October in Baveno
An appointment with innovation in the dermatological field: this is the meaning of National Congress of the Association of Italian Hospital Dermatologists-Venereologists (ADO)which for over sixty years has brought together the major experts in the sector with the aim of promoting updating and discussion on skin diseases. This year’s edition will be held in Baveno, on Lake Maggiore, from 10 to 12 October, and will be a particularly stimulating meeting, given the new diagnosis and therapy opportunities that are appearing in the dermatological field. In the afternoon of Friday 11 October we will also talk about generalized pustular psoriasis (GPP), a rare and serious chronic inflammatory disease of the epidermis whose management is still problematic today.
Often mistakenly thought of as a ‘simple’ aggressive form of psoriasis, GPP is actually a skin disease in its own rightwith pathogenetic mechanisms different from those of plaque psoriasis or other pustular skin diseases. Based on a recent consent document developed by the experts of the European Rare and Severe Psoriasis Expert Network (ERASPEN), the term generalized pustular psoriasis defines “a group of inflammatory skin conditions, relapsing or persistent, characterized by the infiltration of neutrophilic granulocytes into the epidermis to the point of developing pustules primarily sterile and clinically visible, with or without systemic inflammation and with or without concomitant or previous plaque psoriasis”.
The rarity of GPP and its heterogeneous manifestations, sometimes overlapping with those of other skin disorders, pose considerable challenges to the early diagnosis of the disease and timely treatment of patients. Making the picture even more complicated is the intermittent pattern typical of this chronic condition, with sudden and ‘ferocious’ flare-ups which can lead to potentially lethal multi-organ complications. To date, unfortunately, many patients are still forced to wait years before the correct identification of the pathology and, therefore, the possibility of accessing an adequate therapeutic path.
The therapies used to date for the treatment of GPP are in fact indicated for the treatment of other forms of psoriasis. In addition to corticosteroids, administered with the aim of ‘switching off’ inflammation during the acute phase, oral retinoids (acitretin), immunosuppressants (such as ciclosporin and methotrexate) and biological agents intended for the treatment of plaque psoriasis are used. The results, however, are not exceptional, because while in other forms of psoriasis proteins such as tumor necrosis factor alpha (TNF-alpha) and the interleukins IL-17 and IL-23 play an important role, in generalized pustular psoriasis it has been demonstrated that the inflammatory cascade is fundamentally linked to interleukin 36. Fortunately, however, a new drug specifically for GPP has recently been developedwhich could radically change the fate of patients suffering from the disease: it is an anti-interleukin 36 monoclonal antibody, which has already been approved by the European Commission and which it should soon also be available for Italian patients.
In the meantime, let’s remember that the Rare Diseases Observatory (OMaR) provides people suffering from GPP with a guide to Italian centers of expertise in the diagnosis and management of the disease. Created by OMaR with the patronage of APIAFCO (Italian Psoriasis Association Friends of the Corazza Foundation) and thanks to the non-conditioning contribution of Boehringer Ingelheimthe guide has been further updated in recent days, with the addition of new centers, ed It can be consulted online or downloaded in PDF format.
