<\/span><\/h2>\nDarier's disease is rare genetic<\/span> disorder predominantly manifested by scaly<\/span> or crusted papules<\/span>. I like it benign<\/span> family<\/span> pemphigus (Hailey-Hailey disease), Darier's disease is classified as hereditary<\/span> acantholytic<\/span> skin disease<\/span>.<\/p>\nAlso known as Darier-White disease, Darier's disease was previously called 'keratosis<\/span> follicular ", but this is incorrect because squamous papules do not arise from hair<\/span> follicles<\/span>.<\/p>\n<\/span>Who gets Darier's disease?<\/span><\/h2>\nThe onset of skin changes is usually in adolescence and the disease is usually chronic<\/span>. Darier's disease is inherited in a autosomal<\/span> dominant pattern, meaning that a single gene<\/span> Past of a parent causes the condition. The chance that a child will inherit the abnormal gene if one parent is affected is 1 in 2 (50%), but not everyone with the abnormal gene will develop symptoms of the disease.<\/p>\n\n
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<\/span>What is the cause of Darier's disease?<\/span><\/h2>\nThe abnormal gene in Darier's disease has been identified as ATP2A2<\/em>, found in chromosome<\/span> 12q23-24.1. This gene codes for SERCA enzyme<\/span> or pump (sarcoendoplasmic reticulum calcium-ATPase) that is required to transport calcium within the cell. The exact mechanism by which this abnormal gene causes the disease is still under investigation, but it appears that the way skin cells come together may be affected. Skin cells (keratinocytes<\/span>) are held together through structures called desmosomes<\/span> and it seems that desmosomes don't assemble properly if there isn't enough calcium.<\/p>\n<\/span>How is Darier's disease diagnosed?<\/span><\/h2>\nDarier's disease is usually diagnosed by its appearance and family history, but is often mistaken for other skin problems.<\/p>\n
Diagnosis may require skin biopsy<\/span>. the histology<\/span> is characteristic, known as focal<\/span> acantholytic dyskeratosis<\/span> associated with varying degrees of papillomatosis<\/span>. The pathological features are similar to transient acantholytic dermatosis (Grover's disease).<\/p>\n<\/span>What are the clinical features of Darier's disease?<\/span><\/h2>\nThe symptoms and signs of Darier's disease vary markedly between individuals. Some have very subtle signs that are asymptomatic<\/span> and found only on careful inspection. Others have extensive injuries that can cause considerable distress to the affected individual. In an affected person, the severity of the disease can fluctuate over time. <\/p>\nSkin lesions are characterized by persistent, greasy and scaly papules. Affected sites include:<\/p>\n
\n- \nSeborrheic<\/span> areas of the face (margins of the scalp, forehead, ears, around the nostrils and the sides of the nose, eyebrows and beard area)<\/li>\n
- Scalp and neck<\/li>\n
- Central chest and back<\/li>\n
- Skin folds, such as armpits, groin, under the breasts and between the buttocks.<\/li>\n<\/ul>\n
The papules have a firm, hard feel like thick sandpaper and can be skin-colored, yellow-brown, or brown. If several of the small papules grow together, they can form larger warty<\/span> lesions that can smell quite deep inside the skin folds. There may be a very scab eruption<\/span> similar to seborrheic dermatitis<\/span>. <\/p>\nAtypical<\/span> Darier's disease presentations are common.<\/p>\n\n- Flat freckle-like lesions<\/li>\n
- Blistered papules<\/li>\n
- Large, raised and warty lesions<\/li>\n
- A linear<\/span> pattern, with papules following the embryonic development lines of the skin (segmental<\/span> distribution<\/span>)<\/li>\n<\/ul>\n
Acne conglobata (cystic<\/span> acne) is associated with Darier's disease in some people. <\/p>\n\n<\/span>Darier's disease<\/span><\/h5>\n<\/section>\n\n\n
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Darier disease in the middle of the back<\/p>\n
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Scalp disease<\/p>\n
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Facial cysts<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Verruciform acrokeratosis<\/span><\/h3>\nWarty papules on the back of the hands are known as verruciform acrokeratosis. Other signs may include:<\/p>\n
\n- Small holes in the palms and soles.<\/li>\n
- Bleeding under the skin <\/li>\n<\/ul>\n
A located<\/span> The variant of Darier's disease, in which the papules on the hands and feet are the main feature, is called Hopf's acrokeratosis verruciforme.<\/p>\nMost patients with Darier's disease will have broad white and reddish longitudinal stripes on some or all of their nails. A V-shaped notch on the free edge of the nail also suggests Darier's disease.<\/p>\n\n<\/span>Darier hand and foot disease<\/span><\/h5>\n<\/section>\n\n\n
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Nail disease<\/p>\n
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Nail disease<\/p>\n
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Holes Palmar<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>Injuries affecting the mucous<\/span> membranes<\/strong><\/span><\/h3>\nMucous membranes<\/span> are the red, wet linings of body surfaces (mouth, esophagus, rectum, vulva<\/span>, vagina) Patients with Darier's disease may have a white cobblestone pattern of small papules that affect the mucous membranes. Overgum growth can also occur.<\/p>\n\n
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<\/span>\nForecast<\/span> and complications of Darier's disease<\/span><\/h2>\nMost affected patients develop signs of Darier's disease before age 30.<\/p>\n