Erythema marginatum as a precursor of hereditary angioedema

L.L. Lazarenko^1,2, A.M. Milichkina¹, M.A. Saitgalina¹, Yu.V. Ostankova¹, T.P. Sess², I.V. Khamitova¹, A.A. Totolyan^1,2 

¹Pasteur Research Institute of Epidemiology and Microbiology, St. Petersburg

²Pavlov First Saint-Petersburg State Medical University, St. Petersburg 

The presented clinical case is intended to familiarize with the clinical manifestations of hereditary angioedema, including prodromal symptoms, such as erythema marginatum. The disease pattern is the estrogen-dependent nature of edema (debut during pregnancy, condition deterioration during the use of oral contraceptives) and the presence of erythema marginatum. The female patient with a diagnosis of "allergy of unclear etiology" participated in a follow-up for a long period of time. Difficulties in the differential diagnosis of angioedema and erythema marginatum delayed the correct diagnosis for 6 years. Laboratory diagnostics and genetic study were used to establish the diagnosis. A decrease in the concentration and activity of the C1 complement inhibitor, a missense mutation in exon 7 of the SERPING1 (C1NH) gene was revealed. Besides, it was found that the patient’s mother suffered from similar edema from her youth. Prescribed treatment with a bradykinin B2 receptor antagonist (icatibant) contributed to the rapid resolution of angioedema and erythema marginatum. A carefully collected medical history, including family, allergological, pharmacological, detailed examination, the use of all kinds of laboratory diagnostics, including genetic studies, helped in the diagnosis of hereditary angioedema.

Keywords: hereditary angioedema, erythema marginatum, prodromal symptoms, diagnosis, genetic studies, treatment, icatibant.

For citation: Lazarenko L.L., Milichkina A.M., Saitgalina M.A. et al. Erythema marginatum as a precursor of hereditary angioedema. RMJ. 2022;8:39–42.