Pyoderma gangrenosum, pathogenetic associations, clinical presentation, and course features in children

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Pyoderma gangrenosum (PG) is a rare chronic inflammatory neutrophilic dermatosis with a complex and poorly understood etiology and pathogenesis characterized by specific skin lesions with evolutionary polymorphism. We present an up-to-date review on the history of study, etiology, pathogenesis, clinical presentation, diagnostic criteria, differential diagnosis, pathology, and treatment of the disease. PG rash presents as sterile pustules, ulcers at different stages of development, and thick multilayer crusts. Rash elements tend to coalesce rapidly into irregularly shaped vegetative plaques. Involvement of mucous membranes and association with internal organ diseases are common. Depending on the clinical presentation, there are several main forms of PG: classical (ulcerative), pustular, pyodermatitis-pyostomatitis vegetans, bullous and superficial granulomatous. PG is common in some autoinflammatory syndromes: PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne), PASS (pyoderma gangrenosum, acne, suppurative hidradenitis, and seronegative spondyloarthritis), PASH (pyoderma gangrenosum, acne, and suppurative hidradenitis), and PAPASEH (pyogenic sterile arthritis, pyoderma gangrenosum, acne, and suppurative hidradenitis). The verification of the disease is challenging due to the variety of clinical manifestations. The prognosis of PG is determined by timely diagnosis, the presence/absence of internal organ diseases, and the early administration of adequate treatment to prevent the complications. The presented material warrants the interdisciplinary combination of primary care physicians and specialists in the management of patients with PG.

Keywords:

pyoderma gangrenosum

pathogenetic associations

clinical presentation

course features in children

Authors:

Elkin V.D.

Perm State Medical University

ORCID: 0000-0003-4727-9531

Sedova T.G.

E.A. Vagner Perm State Medical University

ORCID: 0000-0002-2660-0536

Plotnikova E.V.

City Children Multidisciplinary Specialized Clinical Center of High Medical Technologies

Zhukova A.A.

Perm State Medical University named after Academician E.A. Wagner

Received:

08.04.2021

Accepted:

15.02.2022

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