Clinical Disease Patterns in a Regional Swiss Cohort of 34 Pyoderma Gangrenosum Patients

Abstract

Background/Aim: Pyoderma gangrenosum (PG) is a rare, neutrophilic dermatosis often associated with an underlying disease, and clinical data or larger studies are rare. Methods: In this retrospective study, disease characteristics, clinical manifestations, and treatment response were evaluated in a Swiss cohort of PG patients. Results: In participating centers, 34 cases (21 females) of PG were analyzed based on clinical and histological presentation between 2002 and 2012. The mean age at diagnosis was 61.2 years; 50% of the patients experienced only 1 episode of PG. In 13 cases (out of 20), recurrences occurred during PG therapy; 64.1% showed only 1 lesion simultaneously. The predominant localization was the lower limb (67%). The lesions were disseminated in 26.6%. At the time of diagnosis or recurrence, the mean diameter was 37.6 mm and the mean ulcer size was 10.3 cm². C-reactive protein (CRP) was elevated in 73.2%; leukocytosis was present in 58.9% and neutrophilia in 50.9%. At least 1 associated comorbidity was present in 85% (the most prominent being cardiovascular disease). The most often used systemic treatments were steroids (68.3%), cyclosporine A (31.7%), dapsone (31.7%), and infliximab (13.3%), and the most often used topicals were tacrolimus 0.1% (48.3%) and corticosteroids (35%). PG healed completely at discharge in 50.8%. The average time to diagnosis was 8 months, and the mean duration to healing was 7.1 months. Conclusion: PG is a difficult-to-diagnose skin disease. Here, markers for inflammation such as CRP, leukocytosis, and neutrophilia were elevated in 50-73% of the PG patients.

Pyoderma Gangrenosum in an African American Male Initially Presenting as Sepsis

“…The most used systemic drugs include steroids, cyclosporine, dapsone, and infliximab. In most cases, systemic therapy utilized one drug, with 25% requiring a combination of two treatments [ 9 , 10 ]. In this case, since the lesion was too large to adequately treat with topical therapy alone, oral prednisone was used for systemic therapy.…”

Une lesion cutanée persistante non cicatricielle depuis 3 ans pyoderma gangrenosum

“…Cette dernière peut précéder le PG, mais celui-ci peut aussi la révéler ou la précéder. L'évolution des lésions cutanées est habituellement indépendante de celle de la maladie sous-jacente [7] (tumeurs solides,..). Près de la moitié des cas concernent des patients présentant des pathologies inflammatoires systémiques ou onco-hématologiques.…”

“…À la clinique, le PG se présente initialement par des pustules inflammatoires coalescentes, qui fusionnent progressivement en laissant apparaitre un ulcère nécrotique, avec des bords hypertrophiques bien délimités, de couleur violacée [4] et qui ne guérit pas avec les traitements conventionnels. De nombreux auteurs décrivent un phénomène de pathergie, l'ulcère apparaissant à la suite d'un traumatisme mineur ou à l'emplacement d'un site chirurgical récent [7]. Cette description va parfaitement de pair avec l'observation clinique faite chez notre patient.…”

Paraneoplastic pyoderma gangrenosum associated with adenocarcinoma of the rectosigmoid junction: a case report

“…PG is a dermatosis that can occur in isolation. However, in more than 75% of cases, it is associated with an underlying pathology, including inflammatory bowel disease; endocrine, hematological, and rheumatological disorders; and nonhematological neoplasia [4]. Few cases of PG related to colorectal cancer have been described.…”

“…Pyoderma gangrenosum (PG) is a rare, idiopathic, inflammatory, neutrophilic dermatosis that is generally characterized by recurrent sterile skin ulceration, with rare extracutaneous involvement [1–3]. Because of its clinical polymorphism related to its different clinical forms, its similarity to other infectious or vascular skin disorders, and its nonspecific histological nature, PG is often a diagnosis of elimination [4]. It can be associated with underlying systemic diseases, the most common of which are inflammatory bowel disease, rheumatic diseases, and malignant blood diseases [5].…”