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Kanpur girl gets rare incurable Darier's skin disease

A 20-year-old Kanpur girl was detected with the deadly, incurable skin disease — Denmark, Slovenia and Scotland Darier disease (DAR or Dyskeratosis Follicularis or Keratsis Follicularis).

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Clinical photograph reveals hyperkeratotic vegetative and hyperpigmented papules with a zosteriform pattern (Representational Image for Darier's skin disease)
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A 20-year-old Kanpur girl was detected with the deadly, incurable skin disease — Denmark, Slovenia and Scotland Darier disease (DAR or Dyskeratosis Follicularis or Keratsis Follicularis).

Shalini, who went to GSVM Medical College in Kanpur to treat skin rashes, was diagnosed with DAR after more than a month’s pathological research. So far, about 15 cases of DAR have been reported in India and this is the first case in Uttar Pradesh. While it doesn't kill, the incurability of the disease is reportedly debilitating.

Mahnendra Singh, the head of the Pathology department at GSVM, said, initially they could not detect the girl’s disease, which had given her dark red crusty patches. “We conducted pathological tests for over a month and consulted our counterparts abroad and finally came to the conclusion that the she was carrying Darier skin disease.”

Mostly, youth in the age group of 16-30 years suffer from the disease. DAR, in the worst case, can lead to the formation of pus, too. Fragility of fingernails, deformity and vertical striations are found in those in the advanced stages.

The disease is genetically inherited from parents but high humidity, stress or wearing tight-fitting clothes can also work as triggers. Worldwide, researchers are struggling to find a cure for the disease, which is most common in East England, Scotland, Slovenia, and Denmark.

It usually affects the chest, neck, back, ears, forehead, and groin and may spread to other areas. Patients suffering from DAR usually go into deep depression and some have reportedly tried committing suicide, too. Patients are usually given neuro-psychiatric treatment to tackle the trauma and fear of social stigma commonly associated with it.

Prof Singh said that they had earlier thought that the girl had developed some strain of psoriasis and had referred her to the surgery department. “A biopsy revealed that it wasn't so. That is when we began conducting various pathology tests.”

“DAR is not contagious. So, we don't know how she contracted DAR or who the carrier was. These are some of the questions we are still trying to find an answer to,” he said.

 

Precaution is the only known prevention and dermatologists generally recommend patients to avoid extremely humid conditions or exposure to direct sunlight.

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