Pyoderma gangrenosum: challenges and solutions

Abstract

Pyoderma gangrenosum (PG) is a rare disease, but commonly related to important morbidity. PG was first assumed to be infectious, but is now considered an inflammatory neutrophilic disease, often associated with autoimmunity, and with chronic inflammatory and neoplastic diseases. Currently, many aspects of the underlying pathophysiology are not well understood, and etiology still remains unknown. PG presents as painful, single or multiple lesions, with several clinical variants, in different locations, with a non specific histology, which makes the diagnosis challenging and often delayed. In the classic ulcerative variant, characterized by ulcers with inflammatory undermined borders, a broad differential diagnosis of malignancy, infection, and vasculitis needs to be considered, making PG a diagnosis of exclusion. Moreover, there are no definitively accepted diagnostic criteria. Treatment is also challenging since, due to its rarity, clinical trials are difficult to perform, and consequently, there is no "gold standard" therapy. Patients frequently require aggressive immunosuppression, often in multidrug regimens that are not standardized. We reviewed the clinical challenges of PG in order to find helpful clues to improve diagnostic accuracy and the treatment options, namely topical care, systemic drugs, and the new emerging therapies that may reduce morbidity.

Neutrophilic dermatosis of the dorsal hands (acral Sweet syndrome) with predominantly lymphocytic dermal infiltrate

“…The previously used term “pustular vasculitis of hands” was revised after the recognition that vasculitis was an inconsistent finding and of secondary importance in its diagnosis . In contrast to PG, NDDH does not cause large ulcers, although histopathologic features in the adjacent, non‐ulcerated PG lesions may be similar to NDDH …”

Successful treatment with oral methylprednisolone and cyclosporine for refractory pyoderma gangrenosum in children: Report of a case and review of the literature

“…Pyoderma gangrenosum affects about 4% of children and ranks among the most painful skin disorders . The lesions of PG are characteristic, beginning with little pustules that progress to enlarging ulcers . It often requires combination therapies, especially in the pediatric population.…”

“…1 The lesions of PG are characteristic, beginning with little pustules that progress to enlarging ulcers. 3 It often requires combination therapies, especially in the pediatric population. Till now, the treatment has been almost based on case reports, so we summarized the therapies of PG in children here.…”

Topical timolol for the treatment of pyoderma gangrenosum

“…Nevertheless, localised and mild PG may be effectively controlled using topical agents without incurring the side effects associated with systemic treatments. In most cases, the preference is for corticosteroids and calcineurin inhibitors 2 3…”