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ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
ANSWER
The correct answer is all of the above (choice “e”).
DISCUSSION
Most cases of dermatomyositis, which the patient’s presentation and lab results suggested, require nerve conduction studies, a check of serum aldolase levels, and skin and muscle biopsies to complete the work-up. However, the arrival at a diagnosis is only the first step.
Patients with dermatomyositis, particularly those older than 60, require evaluation for occult malignancy. There is evidence that the body’s immune response to the cancer is what drives the disease process. Hence the need for the studies listed, looking for breast, lung, and gastrointestinal cancers especially.
Dermatomyositis is thought to be an inflammatory myopathy, possibly driven by autoimmune factors. It is rare (about 1 to 22 per 100,000) and affects women more than men.
The “sunburn” rash is typical, especially on the face, chest, and dorsal hands, and usually clears completely when the cancer is found and cured. Other common findings include elevated creatine kinase, hand rashes (known as Gottron’s papules), and dystrophic calcification in skin and/or joints.
TREATMENT
Aside from addressing a possible malignancy, treatment of dermatomyositis usually starts with glucocorticoids, eventually tapered and replaced by steroid-sparing agents such as azathioprine or cyclosporine. These drugs have dramatically increased the chances of survival and eventual cure.
It’s common for the photosensitivity to persist long after the myositis has resolved, which is why sunscreen and other sun-protective measures are advised.
A 60-year-old woman was referred to dermatology for evaluation of “sunburn.” The rash was painful and unrelieved by topical medications, including class IV steroid creams. The redness was tender and warm to touch.
The rash had been present for months. During this period, the patient also had grown increasingly weak, leading her to quit her job. In the clinic, she was unable to stand from a seated position without difficulty. She reported no other health concerns and had quit smoking 5 years previously, after 30 years.
On examination, diffuse blanchable macular erythema on the patient’s face and chest was immediately observed. There was also an odd rash, composed of hundreds of tiny confluent papules, concentrated over the interphalangeal joints and dorsal hands. These too were warm and tender to touch. Most of her cuticles were peeling off; closer examination under magnification revealed tortuous capillaries on the distal cuticles of several fingers.
Bloodwork revealed a creatine kinase level slightly greater than 1000 U/L, and a positive antinuclear antibody, dilution unknown.
