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Darier disease

What are the symptoms?

The characteristic features of Darier disease are firm, rather greasy, rough papules, which are skin coloured, yellow-brown or brown. The papules may join up in certain moist areas of the body such as the armpits, groin, or under the breasts to form large, sometimes thick plaques. When this occurs, the affected skin may smell unpleasant. The smell is caused by bacteria growing in the rash and does not mean that the skin has not been washed. In some individuals large warty masses cause embarrassment. The psychosocial consequences of the papules may be one of the main difficulties faced by individuals with Darier disease.

Many individuals become aware of the signs of Darier disease before the age of thirty years and in the majority, it starts between the ages of ten to twenty years. At first papules tend to appear on an individual's chest, neck or upper back and many papules are freckle-like. In addition, an individual's scalp, forehead, neck and the skin around the ears may be scaly and itchy. Occasionally, small spots may form in the mouth, which gives the roof or sides of the mouth a rough feeling.

Papules are initially skin-coloured but become darker and more greasy and crusted over time. Darier disease tends to worsen with exposure to sunlight, typically in the summer. Thus affected regions may improve during the winter. The itch of Darier disease may be exacerbated by wool or nylon clothing. Some women may notice that it worsens around the time of their period.

Individuals with Darier disease may have dry and brittle fingernails that sometimes appear 'bitten'. The nails often have red and white longitudinal bands with a V-shaped nick at the free margin of the nail. Wart-like bumps on the backs of the hands may be present in childhood and pits or small 'corns' may appear on the palms of the hands or on the soles of the feet. Some individuals notice that the condition improves or stabilises as they get older, whilst others report that the condition slowly gets worse.

Individuals may be susceptible to skin infections with the herpes simplex virus, otherwise known as the 'cold sore' virus. This does not mean that Darier disease is contagious - it is not. If the condition suddenly worsens and becomes more painful, then this may be a sign of herpes simplex infection. In these instances, it is recommended that individuals contact their GP or dermatologist.

Darier disease may cause depression and in some families has been linked to learning difficulties.

View Background Background  |  What are the causes? View What are the causes?

Medical text written November 2003 by Contact a Family. Approved November 2003 by Dr S Burge. Last updated April 2006 by Dr S Burge, Assistant Director of Clinical Studies and Consultant Dermatologist, Churchill Hospital, Oxford, UK.

 

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