Understanding Dermatomyositis, the disease that claimed Dangal star's life

Suhani Bhatnagar, known for her role in the movie 'Dangal,' passed away last Friday at the age of 19. Following her demise, her parents disclosed the cause of her untimely death.

In an interview with Press Trust of India, Suhani's parents shared that the young actress had been battling a rare inflammatory condition known as Dermatomyositis, which led to her passing. This event has shed light and brought widespread attention to the rare autoimmune disease called Dermatomyositis. 

Dermatomyositis, an uncommon autoimmune disorder, presents a unique challenge in healthcare. According to the National Institute of Environment Health Sciences, scientists have identified more than 80 types of autoimmune diseases. Often misunderstood due to its rarity and complex symptoms, this condition requires comprehensive understanding and awareness to ensure timely diagnosis and effective management.

 

What is Dermatomyositis?

Dermatomyositis is a rare inflammatory disease that affects the skin and muscles. It falls under the category of idiopathic inflammatory myopathies, a group of disorders characterised by muscle inflammation. This condition can impact individuals of any age, but it most commonly appears in adults between the ages of 40 and 60 and children between 5 and 15 years old. While the exact cause of Dermatomyositis remains elusive, it is widely believed to result from an abnormal immune response, wherein the body's immune system mistakenly attacks its tissues.

Underlying Symptoms

The main symptom of Dermatomyositis is a distinctive skin rash, which often precedes or occurs alongside muscle weakness. It typically manifests with distinctive skin abnormalities. Patients may develop a characteristic rash, known as heliotrope rash, on the eyelids, along with a reddish or purplish rash on the face, neck, chest, shoulders, and other areas exposed to the sun. Additionally, a rash on the knuckles, elbows, knees, and other bony prominences, termed Gottron's papules, with swelling, is commonly observed. In addition to the skin rash, individuals with Dermatomyositis may experience muscle weakness, particularly in the proximal muscles of the shoulders, hips, and thighs, which is a hallmark feature of Dermatomyositis. This weakness may manifest gradually, leading to difficulties in activities such as climbing stairs, rising from a seated position, or lifting objects. 

In addition to skin and muscle involvement, Dermatomyositis may also present with systemic symptoms such as fatigue, weight loss, fever, and difficulty swallowing (dysphagia). In some cases, like in Suhani's situation, Dermatomyositis may be associated with complications affecting other organs and systems, including interstitial lung disease, myocarditis (heart muscle inflammation), and gastrointestinal involvement. The Dangal actress developed fluid in her lungs, which led to their failure days before her demise.

Causes and risk factors

While the precise cause of Dermatomyositis remains unknown, several factors are believed to contribute to its development. It is considered an autoimmune disease, wherein the body's immune system mistakenly attacks its own tissues. Although Dermatomyositis is not considered a hereditary condition, certain genetic factors may predispose individuals to develop the disease. Certain environmental factors, such as infections, medications, and exposure to ultraviolet (UV) radiation, have been implicated as potential triggers for Dermatomyositis in susceptible individuals. Additionally, the condition tends to be more prevalent in females than males.

Diagnosis and Evaluation

Diagnosing Dermatomyositis often involves a combination of clinical evaluation, laboratory tests, imaging studies, and muscle biopsy. A healthcare provider will assess the characteristic skin rash and muscle weakness while also conducting blood tests to check for elevated muscle enzymes and specific autoantibodies associated with the condition. Electromyography (EMG) and muscle biopsy may be performed to evaluate muscle function and examine tissue under a microscope for signs of inflammation.