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Related Concept Videos

Rheumatic Heart Disease III: Medical Management01:21

Rheumatic Heart Disease III: Medical Management

Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...
Peripheral Artery Disease III: Interprofessional Care01:27

Peripheral Artery Disease III: Interprofessional Care

Peripheral Artery Disease (PAD) is characterized by narrowed arteries that diminish blood flow to the extremities. Effective management of PAD requires an interprofessional approach involving various healthcare professionals. The critical aspects of interprofessional care for PAD patients focus on risk factor modification, drug therapy, exercise therapy, nutrition therapy, critical limb ischemia care, and interventional radiology and surgical procedures.The primary treatment goal for PAD...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
Aortic Regurgitation III: Medical Management01:25

Aortic Regurgitation III: Medical Management

Aortic regurgitation (AR) is when the aortic valve does not close or seal properly, leading to backward blood circulation from the aorta into the left ventricle during diastole. Common causes of AR include rheumatic heart disease, congenital valve defects, and aortic root dilation. Managing AR requires a multifaceted approach to alleviate symptoms, preserve left ventricular function, and address the underlying cause of the regurgitation. Patients with symptomatic AR or significant left...
Atherosclerosis III: Management01:26

Atherosclerosis III: Management

Management of atherosclerosis involves an integrated strategy encompassing pharmacological treatment, surgical interventions, lifestyle changes, and nutrition therapy to address the multifactorial nature of the disease.Pharmacological TherapyA cornerstone of atherosclerosis management is the use of pharmacological agents. Statins, such as atorvastatin, are pivotal in inhibiting HMG-CoA reductase, an enzyme that catalyzes an initial step in cholesterol synthesis in the liver. This reduction in...
Rheumatic Heart Disease IV: Nursing Management01:20

Rheumatic Heart Disease IV: Nursing Management

AssessmentA comprehensive assessment is essential in managing a patient with rheumatic heart disease (RHD). Begin with obtaining a detailed medical history, including recent streptococcal infections, a history of rheumatic fever, or previously diagnosed rheumatic heart disease. Assess the patient for symptoms such as fever, chest pain, widespread joint pain (arthralgia), tachycardia, pericardial friction rub, muffled heart sounds, heart murmurs, peripheral edema, subcutaneous nodules, and...

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Updated: Jul 16, 2026

Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus
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Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus

Published on: May 16, 2025

Consensus Recommendations for Management of Darier Disease: A Practical Approach.

Cristina Has1, Valentina Ruffo di Calabria2, Marzia Caproni2

  • 1Department of Dermatology, Expert Center of ERN-Skin, Medical Center University of Freiburg, Freiburg, Germany. cristina.has@uniklinik-freiburg.de.

Dermatology and Therapy
|July 15, 2026
PubMed
Summary

Darier disease (DD) is a rare genetic skin disorder impacting quality of life. This study offers practical recommendations for diagnosis and management, emphasizing individualized care and future research for novel treatments.

Keywords:
Acantholytic disorderComplicationDarier diseaseDiagnosticEpidermal differentiation disorderRetinoidsSystemic anti-inflammatory therapiesTherapy

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Last Updated: Jul 16, 2026

Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus
05:39

Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus

Published on: May 16, 2025

Area of Science:

  • Dermatology
  • Genetics
  • Rare Diseases

Background:

  • Darier disease (DD) is a rare, autosomal dominant genodermatosis stemming from ATP2A2 gene variants.
  • Characterized by chronic skin lesions and extracutaneous issues, DD significantly impairs patients' quality of life.
  • Onset typically occurs post-puberty, influenced by environmental factors and exhibiting variable expressivity.

Purpose of the Study:

  • To establish evidence- and consensus-based recommendations for managing Darier disease patients.
  • To guide healthcare professionals, especially in non-expert settings, on DD patient care.
  • To identify and discuss emerging therapeutic strategies for Darier disease.

Main Methods:

  • Systematic literature review and expert consensus from the European Reference Network for Rare and Undiagnosed Skin Disorders (ERN-Skin).
  • Inclusion of evidence from case reports, case series, and mechanistic studies.
  • Formulation of guidance for symptomatic and advanced targeted therapies.

Main Results:

  • A total of 68 recommendations were agreed upon for DD management.
  • Diagnosis relies on clinical presentation, family history, histopathology, and ATP2A2 genetic testing.
  • Management includes trigger avoidance, topical treatments (corticosteroids, retinoids), systemic therapies for severe cases, and physical modalities for resistant lesions.

Conclusions:

  • DD is a chronic genetic disorder significantly affecting quality of life, necessitating multidisciplinary and individualized management.
  • Recommendations provide a framework for diagnosis, management, and follow-up.
  • Further research, clinical trials, and registries are crucial for advancing therapeutic strategies and developing novel treatments.