Darier's disease (DAR) is an inherited skin disorder that presents with multiple greasy, crusting, thick brown bumps that merge into patches. It is an autosomal dominant disorder discovered by French dermatologist Ferdinand-Jean Darier. Darier's is characterized by dark crusty patches on the skin that are mildly greasy and that emit a strong odor. These patches, also known as keratotic papules, keratosis follicularis, or dyskeratosis follicularis, most often appear on the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. Mild forms of the disease are the most common, consisting solely of skin rashes that flare up under certain conditions such as high humidity, high stress, or tight-fitting clothes. Short stature, when combined with poorly-formed fingernails that contain vertical striations, is diagnostic even for mild forms of DAR. Symptoms will usually appear in late childhood or early adulthood between the ages of 15 to 30 years and will vary over the course of one's life. Signs and symptoms Clinical symptoms of the disease: Seborrhoeic areas This is defined as areas where excess oil and sebum is released. Overall greasy or scaly skin either in the central chest and back or in the folds of the skin. Fragile or poorly formed fingernails Nail disease leading to V-shaped nicks at the edge of the nail. Rash that covers many areas of the body The rash is often associated with a strong unpleasant odor The rash can be aggravated by heat, humidity, and exposure to sunlight. Mucosal manifestation White cobblestone pattern of small papules Overgrowth of gums Usually affects the mouth, esophagus, rectum, vulva, vagina Oral symptoms can be diagnosed by a routine dental examination Other symptoms and their overall prevalence in the affected population: In 80 to 90% of patients Acrokeratosis verruciformis Acrokeratosis is characterized by several small wart-like and flat-topped bumps that line the skin on typically the hand and feet. Hypermelanotic macule Patches on the skin that contain excess pigment, they often appear as dark patches in the skin. Pruritus Itching Subungual hyperkeratotic fragments Thickened skin that is often discolored, under nails, on either hands or feet. Palmar pits Usually red in color, they are pits or depressions in the palms or soles of the hands and feet. In 30 to 79% of patients Abnormal hair morphology Acne conglobata Typically described as cystic acne Genetics Mutations in a single gene, ATP2A2, are responsible for the development of Darier's