Healthy Soch
It is a rare disorder, inherited in an autosomal dominant pattern
New Delhi, December 4, 2017: Recently, a case of Darier’s disease was reported from a city in North India. So far, about 15 cases have occurred in India, with this being the first in Uttar Pradesh. The disease is so rare that it affects only one out of one lakh people. Although the disease does not kill, it can be potentially debilitating for the person in question and put them on lifelong precautionary treatment.
Darier disease is a skin condition characterized by wart-like blemishes on the body. The blemishes are usually yellowish in color, hard to the touch, mildly greasy, and can emit a strong odor. The most common sites for blemishes are the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. The mucous membranes can also be affected, with blemishes on the roof of the mouth (palate), tongue, inside of the cheek, gums, and throat.
Speaking about this, Padma Shri Awardee Dr K K Aggarwal, National President Indian Medical Association (IMA) and President Heart Care Foundation of India (HCFI) and Dr RN Tandon – Honorary Secretary General IMA in a joint statement, said, “The wart-like blemishes in this disease usually appear in late childhood to early adulthood. Without precautionary measures, it can become more severe over time with the affected people experiencing flare-ups alternating with periods when they have fewer blemishes. The appearance of the blemishes is influenced by environmental factors such as heat and humidity; UV light; minor injury or friction; and ingestion of certain medications. At times, people with Darier disease may have neurological disorders; and issues with learning and behavior as well. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder.”
Itching is very common in those with this condition. The affected skin may smell unpleasant, particularly in moist areas. This is probably caused by increased numbers of ordinary skin bacteria growing in the affected skin.
Adding further, Dr Aggarwal, said, “The diagnosis of Darier’s disease can often be made on the appearance of the rash and the fact that it runs in families. To confirm it, a small sample of skin (a biopsy) can be removed under a local anesthetic and examined under the microscope in the laboratory. Genetic testing to identify a mutation in the ATP2A2 gene can be used to confirm the diagnosis as well.”
Some precautions to be taken for those with this disease include the following.
- Avoid stress, excessive humidity, heat and tight clothes and use moisturizers, sun block and wear the right type of clothes which absorb sweat and keep the body cool.
- For localized lesions, dermabrasion can be done. Topical retinoids are also prescribed and are beneficial.
- If the patient has secondary bacterial infection, then antibiotics are prescribed. Antiviral medications are prescribed if the patient has herpes simplex.
- For severe symptoms, oral retinoids can be prescribed, but these medications have serious side effects and need to be used with caution. Topical or oral antibiotics can be prescribed during flare ups.
- Using sunscreen and taking vitamin C tablets helps in preventing flare ups in some patients.
- Patients should also maintain good hygiene to prevent flare ups.
