Dermatomyositis: What It Is and How It Affects the Skin
When an ailing Maria Callas–world-renowned soprano–slipped out the back door of an opera house in Rome on January 2,1958, she knew her fans would be deeply unhappy. She probably did not know the magnitude of the incoming storm of outrage. It was the opening night of the Rome Opera Season, and Callas was the star in a performance of Bellini’s Norma. Callas had performed a single act when she became too weak and ill to continue.
In the following days, the arts journals and tabloids went wild. Callas’ unusually dramatic weight loss a few years earlier had sparked rumors that she’d intentionally ingested tapeworms, an accusation that was revived. Patrons of the arts suggested that spite, not illness, explained her retreat from the performance. The Italian Parliament discussed banning her from performing in state-funded opera houses.
Years later, Callas’ decision to retire at age 42 was met with the same scorn. Her vocal range and control had deteriorated noticeably, and her lung capacity was diminished. The press attributed this to laziness and disinterest; Callas was involved in a tempestuous romance with Greek shipping magnate Aristotle Onassis, and her critics regarded her early retirement as proof that she valued wealth and leisure over her craft.
The gossip did not end when Callas died unexpectedly in 1977. The heart attack that ended her life at the relatively young age of 53 was ascribed to heartbreak over Onassis’ abandonment and subsequent death.
In 2002, Callas’ physician spoke out for the first time; Callas had been diagnosed with a rare disorder called dermatomyositis two years before her death in 1975. This, he said, not heartbreak, contributed to her death.
Dermatomyositis and Maria Callas’ Literal Heartbreak
Dermatomyositis is one example of inflammatory myopathies, a group of rare diseases characterized by inflammation and weakness of the muscles.
Anyone can develop the disorder. However, it is most prevalent in women, and most patients are between 40 and 60 when they are diagnosed. Fevers, rashes, weakness, and weight loss are common symptoms in the early stages of the disorder. Elevated creatinine kinase (CK) is often noted in blood work, indicating damage to skeletal muscles.
Weakness of the skeletal muscles closest to the trunk, such as those in the hips, thighs, shoulders, upper arms and neck, is the most common symptom of dermatomyositis. Both the left and right sides of the body are affected, and weakness tends to worsen gradually. Over time, the distal muscles can be affected.
These muscles can become tender or sore, and weakness can eventually lead to difficulty performing tasks such as lifting the arms or climbing stairs. For long-term patients, this can cause a loss of muscle bulk, or atrophy. In particularly severe cases, contractures, in which joints become permanently fixed in a bent position, can occur.
Dermatomyositis can also affect the muscles involved in singing and speech, making both more difficult–especially in more severe cases. Dysphagia, or difficulty swallowing, is widely noted. The heart muscle can also be affected.
The disorder is often misdiagnosed as systemic lupus erythematous (SLE), and to complicate matters, the two disorders are known to occur together. It is also associated with certain cancers (breast, nasopharyngeal, stomach, ovarian, lung, pancreatic, colorectal, melanoma, and non-Hodgkin’s lymphoma). These cancers are usually diagnosed within the first two years after the onset of dermatomyositis symptoms.
Understanding dermatomyositis allows us to examine Maria Callas’ career and death anew; the unexplained bouts of fever and weakness, the falling vocal range and lack of control, the constricted posture with which she performed towards the end of her career appear less like melodrama and more like symptoms of a progressive, inflammatory muscle disorder.
Dermatomyositis: A Changeable Outlook
Dermatomyositis is chronic and incurable, and while the prognosis for most patients is quite good, for some it is progressive–as seems to have been the case with Maria Callas. The disorder could have contributed to her heart attack directly through inflammation of the heart muscle.
It could also have contributed indirectly; vasculitis, or inflammation of the blood vessels, is a common complication of dermatomyositis. The damage it does to the blood vessels could obstruct blood flow to the heart. Interstitial lung disease could have contributed; so too could long-term use of systemic steroids, which would have been the primary treatment available to Callas.
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