Pyoderma gangrenosum: examination and treatment experience

Abstract

BACKGROUND: Pyoderma gangrenosum is a rare inflammatory skin disease that currently belongs to the group of neutrophilic dermatoses.

AIMS: Development of epidemiological, clinical, laboratory characteristics of patients with pyoderma gangrenosum, as well as the treatment of this disease.

MATERIALS AND METHODS: The study revealed 30 patients, 16 (53%) were women, the average age at the time of diagnosis was 59±16.3 years. The most common localization of rashes was the lower leg 20 patients (67%), lesions of the skin of the face, genitals, and breast rarely noted, in one case, respectively. In 14 patients (47%), two or more ulcers were detected at the same time (max. 9 ulcers). The pathergy phenomenon was positive in 23 patients (77%) of 30. Ulcerative pyoderma gangrenosum occurred in 25 (83%), and extracutaneous pyoderma gangrenosum with lung involvement was identified in one patient. The most common associated disease was rheumatoid arthritis in 6 patients (20%), there were also rare associated diseases, such as hepatitis C two cases, multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, non-Hodgkin’s lymphoma by one observation. Histological examination revealed neutrophilic infiltration of the dermis in 30 patients (100%), and the presence of leukocytoclastic vasculitis in 16 (53%). Complete scarring during the treatment was observed in 22 patients (73%). Relapses during the observation period were observed in 12 patients (40%), the number of deaths was two cases.

CONCLUSIONS: One of the largest case series of pyoderma gangrenosum to date is presented. During the examination of patients, it was found that neutrophilic infiltration of the dermis is a characteristic sign of the disease. Rare comorbidities (multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, hepatitis C, non-Hodgkin’s lymphoma) have been found. A sufficiently large percentage of relapses indicates the need for further research to develop an additional examination method for the purpose of modern diagnostics and justification for the timely prescription of targeted therapy.

Keywords

pyoderma gangrenosum / inflammatory bowel diseases / neutrophilic dermatoses / autoinflammatory diseases / systemic steroids

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Teplyuk N.P., Grabovskaya O.V., Kusraeva D.T., Varshavsky V.A.