Evaluation of the efficacy of treatment for the erythrodermic form of Devergie’s disease: a retrospective 10-year single-center study

Background: Devergie’s disease (DD) is a rare inflammatory papulosquamous skin disorder of unknown etiology, often characterized by the development of erythroderma. Due to the lack of data on the etiology and pathogenesis of DD, treatment remains challenging. Systemic retinoids are the first-line therapy for DD, but they are not always effective. However, existing data on the similarity between the pathogenetic mechanisms of DD and psoriasis suggest that genetically engineered biological agents (GEBAs) may be considered as an adjunctive treatment for DD. Objective: Analysis of the data from patients with the erythrodermic form of DD and evaluation of the effectiveness of systemic therapy and phototherapy in treating this disease.

Materials and methods: The study was conducted at the V.A. Rakhmanov Clinic of Skin and Venereal Diseases, Sechenov University Clinical Hospital No. 2, between May 2014 and May 2024. Fifteen patients over 18 years of age with the erythrodermic form of DD were included in the study. Patients were selected based on archival records according to the following criteria: age 18–80 years, presence of the erythrodermic form of DD (erythroderma index (EI) ≥36). The Dermatological Quality of Life Index (DQLI) was assessed before treatment, as well as 2, 3, and 4 months after treatment. Of the 15 patients with DD, 46.7% (n=7) received acitretin, 20% (n=3) – isotretinoin, 33.3% (n=5) – methotrexate, 20% (n=3) – prednisolone, 6.7% (n=1) – dexamethasone, 13.3% (n=2) – netakimab, 6.7% (n=1) – ustekinumab, and 6.7% (n=1) – phototherapy (PUVA, UVB-311nm). Before treatment, the mean EI was 89±3.5 points, and the DIQI was 21±2.1 points.

Results: On acitretin therapy, patients demonstrated significant regression of the rash, followed by a decrease in the EI and DLQI to a mean of 26±3.2 and 3.7±1.9 points, respectively, demonstrating a positive effect of this drug on the dynamics of DD. For example, one patient (6.67%) underwent phototherapy (PUVA and UVB-311 nm) without achieving a clinical effect. However, our study identified cases of stabilization of the skin process and improvement in disease dynamics with a combination of phototherapy and other treatment methods. Thus, we presented cases of acitretin and phototherapy in combination (Re-PUVA) with a mean EI of 39.6±3.2 points and a DLQI of 5±1, which increases the effectiveness of phototherapy for the disease.

Isotretinoin therapy demonstrated a decrease in the EI and DLQI, demonstrating positive disease dynamics, but without complete regression of the rash. The efficacy of methotrexate and phototherapy as monotherapy for Devergie’s disease remains controversial. In cases of relapses after previous therapy, insufficient response, or resistance to treatment, biological agents were the drug of choice, with efficacy comparable to acitretin. Remission in patients after successful treatment of Devergie’s disease with netakimab and ustekinumab was observed for 18 months. It is worth noting that patient follow-up to assess the long-term results of biological therapy for Devergie’s disease is ongoing.

Conclusion: The obtained data support the use of systemic retinoids as first-line therapy for Devergie’s disease, particularly acitretin, which demonstrated superior efficacy compared to isotretinoin in our patients. Biological therapy may be considered an effective adjunctive treatment for severe and resistant forms of Devergie’s disease, as well as promoting long-term remission and improving quality of life in patients with this disease.