Understanding Darier’s Disease: Causes, Symptoms, and Management

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Darier’s Disease

by Alike Medical Team ∙ Updated on June 13, 2023

Additional names

This group contains additional names: - Darier-White disease - Keratosis Follicularis - Dyskeratosis Follicularis

General

Darier's disease is an inherited skin disorder that presents with multiple greasy, crusting, thick brown bumps that merge into patches. Darier's is characterized by dark crusty patches on the skin that are mildly greasy and that emit a strong odor. These patches most often appear on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear.

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Signs & symptoms

* Seborrhoeic areas * Fragile or poorly formed fingernails * Rash that covers many areas of the body * Mucosal manifestation like White cobblestone pattern of small papules, Overgrowth of gums etc.

Diagnosis

Diagnosis of Darier disease is often made by the appearance of the skin, family history, or genetic testing for the mutation in the ATP2A2 gene. However, many individuals affected by this disorder are never diagnosed, due to the mildness of symptoms in most cases. Mild cases present clinically are minor rashes (without odor) that can become exacerbated by heat, humidity, stress, and sunlight. The symptoms of the disease are thought to be caused by an abnormality in the desmosome-keratin filament complex leading to a breakdown in cell adhesion.

Treatment

Treatment of Darier disease depends on the severity of the presented clinical symptoms. In most minor cases, the disorder can be managed using sunscreen, moisturizing lotions, avoidance of non-breathable clothing, and excessive perspiration. In more severe cases of Darier's disease, hospitalisation may be required to heal affected individuals who display frequent relapse and remit patterns. In less severe cases, signs and symptoms may clear up completely through hygienic interventions. Most patients with Darier's disease live normal, healthy lives. Rapid resolution of rash symptoms can be complicated due to the increased vulnerability of affected skin surfaces by secondary bacterial or viral infections. Epidermal Staphylococcus aureus, human papillomavirus (HPV) and herpes simplex virus (HSV) infections have been reported. In these cases, topical and/or oral antibiotic/antiviral medications may need to be prescribed.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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