Purple Rashes, Fever & Muscle Weakness: What's Dermatomyositis, Rare Disease That Led to 'Dangal' Star's Death?

The passing away of ‘Dangal’ actor Suhani Bhatnagar due to dermatomyositis has brought attention to lesser-known risks of this skin disease which is typically not considered fatal.

Dermatomyositis is a rare autoimmune disease affecting both the skin and muscles, characterised by inflammation leading to muscle weakness and skin rashes. Experts told News18 that it is a very rare disease “occurring in 2-3 people for every 1 lakh population” and one of the most noticeable symptoms is a “distinctive skin rash”.

“This rash often appears on the face, eyelids, knuckles, elbows, knees, chest, and back. It may be reddish or purple and is often described as a “heliotrope” rash,” Dr Priyanka Kharbanda, a rheumatologist at Fortis Hospital, Vasant Kunj, told News18.

Gottron’s papules, which are raised, scaly patches, appear over the knuckles and other bony prominences such as the elbows and knees accompanied by excessive weakness of muscles.

The disease was earlier considered to impact people in the age group of 40 to 60 years old but is now being noted among youngsters and children as well.

“The disease is becoming common among youngsters and children but earlier its onset was seen among people in the 40s or later age group. While management of this disease is slightly difficult among children, a very disciplined life along with regular blood tests and an anti-inflammatory diet helps people live a long life,” Dr Deepali Bhardwaj, dermatologist and anti-allergy specialist, Centre for Skin and Hair, told News18.

How does the body develop the disease?

The exact cause of dermatomyositis is unknown, but it may be due to a viral infection of the muscles, mostly due to a problem with the body’s immune system when the system mistakenly attacks its own muscle and skin tissues.

Other causes, according to Dr Shweta Singhai, rheumatology expert at Bengaluru-based Sakra World Hospital, could be bacterial infections, environmental factors, including UV radiation, air pollutants and very occasionally, vaccinations.

“It can sometimes be caused by drugs like statins which are used to reduce cholesterol,” she said, adding that the disease can be malignant in about 30-40 per cent patients and people need to be alarmed if they notice extreme weight loss. “It is a very rare disease occurring in 2-3 people for every one lakh population.”

The causes of the disease, listed by Gaurav Jain, senior consultant, internal medicine and rheumatology at Dharamshila Narayana Suprspeciality Hospital, involve a combination of genetic predisposition, environmental factors, and immune system dysfunction.

How to spot symptoms?

Some of the common symptoms of dermatomyositis include muscle weakness, skin rash, difficulty swallowing, fatigue, and muscle pain.

Kharbanda from Fortis said that muscle weakness is another key feature of dermatomyositis. It typically affects the muscles “closest to the trunk of the body, such as those in the hips, thighs, shoulders, upper arms, and neck”.

“Weakness may make it difficult to perform everyday activities like climbing stairs, standing from a seated position, lifting objects, or reaching overhead,” she said.

Experts suggest that the affected person finds it difficult to raise the arms above shoulder level and also to get up from a sitting position on a chair or the floor. Weakness in the muscles of the neck can cause the head to drop forward involuntarily, a condition known as “dropped head syndrome”.

Individuals with dermatomyositis may experience difficulty swallowing, which can lead to choking or asphyxiation. Also, people experience profound fatigue that is not relieved by rest and this fatigue can significantly impact daily functioning and quality of life.

Experts also suggest that a low-grade fever may accompany the onset or flare-ups of dermatomyositis.

How can it be treated?

Treatment typically involves medications to suppress the immune system’s abnormal activity, such as corticosteroids, immunosuppressants, and sometimes intravenous immunoglobulin (IVIG) therapy. Physical therapy and exercise are often recommended to help maintain muscle strength and function.

“Early diagnosis and treatment are important in managing the condition and preventing complications,” Kharbanda from Fortis Hospital said.

Dermatologists, rheumatologists and other experts added that the disease proves fatal when it affects crucial organs like the lungs, heart, or respiratory system.

Jain, from Dharamshila Narayana Suprspeciality Hospital, added that survival rates of patients depend on early diagnosis. “Effective treatment significantly improves the outcomes. However, complications such as respiratory and cardiac involvement can pose serious risks.”

Dermatologist Bhardwaj further explained that death generally does not happen because of dermatomyositis but due to complications arising from it such as lung myositis when a person is unable to breathe. “Also, the disease increases the risk of developing cancer.”