Pyoderma gangrenosum – a guide to diagnosis and management

Abstract

Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the spectrum of the neutrophilic dermatoses. There are several subtypes, with 'classical PG' as the most common form in approximately 85% cases. This presents as an extremely painful erythematous lesion which rapidly progresses to a blistered or necrotic ulcer. There is often a ragged undermined edge with a violaceous/ erythematous border. The lower legs are most frequently affected although PG can present at any body site. Other subtypes include bullous, vegetative, pustular, peristomal and superficial granulomatous variants. The differential diagnosis includes all other causes of cutaneous ulceration as there are no definitive laboratory or histopathological criteria for PG. Underlying systemic conditions are found in up to 50% of cases and thus clinicians should investigate thoroughly for such conditions once a diagnosis of PG has been made. Treatment of PG remains largely anecdotal, with no national or international guidelines, and is selected according to severity and rate of progression. Despite being a well-recognised condition, there is often a failure to make an early diagnosis of PG. This diagnosis should be actively considered when assessing ulcers, as prompt treatment may avoid the complications of prolonged systemic therapy, delayed wound healing and scarring.

Dermatological Manifestations in Pediatric Inflammatory Bowel Disease

“…PG is associated with several systemic diseases, such as IBD, leukemia, and immunodeficiency (IgA deficiency and HIV positive status) [ 63 ].…”

“…Cutaneous manifestations of IBD secondary to malnutrition and/or malabsorption refer to lesions appearing as a result of vitamin or nutrient deficiency such as zinc deficiency (acrodermatitis enteropathica), niacin deficiency (pellagra), vitamin C deficiency (scurvy), vitamin K deficiency (purpuric lesions), and vitamin B deficiency (glossitis, angular cheilitis). Dry skin and unspecified eczema may appear secondary to essential fatty acid deficiency; hair and nail abnormalities may appear secondary to amino acid and protein malabsorption [ 63 , 70 ].…”

Immunoglobulin M Paraproteinaemias

“…A variety of dermatological disorders may be associated with paraproteinaemias and these range from relatively benign conditions, such as xanthoderma, to more destructive lesions such as pyoderma gangrenosum [ 100 , 101 ]. Patients presenting to dermatologists with these dermatoses may be screened for paraproteins and referred for a haematology opinion [ 101 ].…”

Wound care, adalimumab, and multidisciplinary approach in a patient affected by PASH syndrome

“…8,9 Different treatments have been proposed for PG patients, including topical agents (potent topical corticosteroids and tacrolimus ointment) and systemic conventional treatments (corticosteroids, cyclosporine, colchicine, sulphasalazine, dapsone, minocycline, apremilast, and thalidomide). 10 There is now a growing body of evidence to support biologic therapy as a treatment of PG, with anti-TNF drugs as a well-known efficient treatment option, while promising data are being obtained from treatment with etanercept, ustekinumab, anakinra, and canakinumab. 10 Moreover, several cases showed the increased efficacy of the combination of different treatments.…”

“…10 There is now a growing body of evidence to support biologic therapy as a treatment of PG, with anti-TNF drugs as a well-known efficient treatment option, while promising data are being obtained from treatment with etanercept, ustekinumab, anakinra, and canakinumab. 10 Moreover, several cases showed the increased efficacy of the combination of different treatments. 11 Here, we reported the case of a PASH syndrome patient successfully treated with a combination of anti-TNF-α (adalimumab) and a local intensive treatment combined with a multidisciplinary approach.…”