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. 2024 Jun 11:104:adv19663.
doi: 10.2340/actadv.v104.19663.

Unmet Needs in Darier's Disease from a Patient's Perspective: Lessons Learnt from the German Registry

Danielle Rogner  1   2 , Laura Heimerl  3 , Tilo Biedermann  3 , Elke Sattler  4 , Alexander Zink  5
Affiliations

Unmet Needs in Darier's Disease from a Patient's Perspective: Lessons Learnt from the German Registry

Danielle Rogner et al. Acta Derm Venereol. .

Abstract

The MDHHgermany registry was initiated to characterize the "real-life" situation of affected individuals with Darier's disease (DD; Morbus Darier, MD) and Hailey-Hailey disease (HH), including their treatment and healthcare. To gain deeper insights into medical care of patients with DD, various aspects such as demographics, subjective symptoms, patient satisfaction with medical care, past and current therapies were explored. Patients with diagnosed DD were included. Subjective symptoms such as itch, pain and burning sensation were assessed. Individual therapy goals were recorded and patients assessed previous/current therapies along with satisfaction of medical care and treatment. A total of 55 patients were recruited; 47 patients were eligible for the analysis. Pruritus was rated the most bothersome symptom. Some 42.6% had not received systemic treatment so far or systemic therapies were rated ineffective (32.6%). Most commonly oral retinoids were prescribed, followed by corticosteroids. Patient satisfaction with medical care and treatment proved to be mediocre. This "real-life" data show an alarming unmet need regarding patients' satisfaction with medical care and treatment, evidenced by the reported lack of disease control. Further studies and interventions are needed to improve the spectrum of available therapies. MDHHgermany provides a foundational platform for future clinical trials, epidemiological studies, and pathophysiological analyses.

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Conflict of interest statement

D.R. has received research grants, honoraria as lecturer and/or consultant, and/or travel grants from Eli Lilly, Novartis, and Pfizer. L.H. has no conflicts of interest to be noted. T.B. has no conflicts of interest to be noted. E.S. has no conflicts of interest to be noted. S.H. has no conflicts of interest to be noted. A.Z. has no conflicts of interest to be noted.

Figures

Fig. 1
Fig. 1
Age at first diagnosis (n = 47) (min: 6 years, max: 53 years). x-axis: age in numbers.
Fig. 2
Fig. 2
Display of assessment of disease severity (n = 44). y-axis: number of patients.
Fig. 3
Fig. 3
Display of the symptom “itch” (n = 45) on a 10-point-scale, 0 = no itch, 10 = worst imaginable itch. x-axis: 0- to-10 point scale; y-axis: number of patients.
Fig. 4
Fig. 4
Bar plot of subjective symptoms pain, itch, burning sensation, and sleep disturbance during the past 3 days (n = 45). y-axis: 0- to-10 point scale.
Fig. 5
Fig. 5
Display of ranking of disease burden (n = 46). x-axis: number of patients.
Fig. 6
Fig. 6
Display of therapeutic goals, more than one answer possible (n = 46). x-axis: number of patients.
See this image and copyright information in PMC

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