An unusual pediatric clinical case of atypical pyoderma gangrenosum

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Abstract

Pyoderma gangrenosum (PG) belongs to the group of neutrophilic dermatoses with unknown etiology and poorly understood pathogenesis. In children, PG is primarily associated with inflammatory bowel diseases (Crohn's disease and ulcerative colitis). By now, about 355 PG cases have been described worldwide, including 15 children with the involvement of oral mucosa. Clinical manifestations of the disease are diverse and depend on the form, stage and associated conditions. Such a rare PG as pyodermatitis-pyostomatitis vegetans manifests by combined lesions of the oral mucosa and skin. The authors present historical data on the investigation of the disease, its etiology, pathogenesis, risk factors, and clinical picture. A description of a rare clinical case of severe form of atypical PG, namely, pyodermatitis-pyostomatitis vegetans in a 10-year-old child, is presented. The unique character of the case is related to the variety of clinical manifestations and the clinical course complicated by the development of keloid and partial scar-related flexion contracture of the upper and lower extremities, the formation of microstoma and cachexia. The association of pyodermatitis-pyostomatitis vegetans with Crohn's disease was found. The lack of awareness of the clinical symptoms of this rare disease has led to diagnostic errors and late diagnosis.

About the authors

T. G. Sedova

Perm State Medical University named after academician E.A. Vagner

Author for correspondence.
Email: sedovca-1978@yandex.ru
ORCID iD: 0000-0002-2660-0536

Tat'yana G. Sedova – MD, PhD, Associate Professor, Department of Dermatology and Venerology

26 Petropavlovskaya ul., Perm, 614000

Tel.: +7 (908) 249 91 99

Russian Federation

V. D. Elkin

Perm State Medical University named after academician E.A. Vagner

Email: fake@neicon.ru
ORCID iD: 0000-0003-4727-9531

Vladimir D. Elkin – MD, PhD, Professor, Head of the Department of Dermatology and Venerology

26 Petropavlovskaya ul., Perm, 614000

Russian Federation

A. A. Zhukova

Perm State Medical University named after academician E.A. Vagner

Email: fake@neicon.ru
ORCID iD: 0000-0003-1750-3360

Anastasiya A. Zhukova – MD, Assistant, Department of Dermatology and Venerology

26 Petropavlovskaya ul., Perm, 614000

Russian Federation

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Copyright (c) 2020 Sedova T.G., Elkin V.D., Zhukova A.A.

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