A 67-year-old Caucasian man presented to our clinic with a 4-month history of erythema and scaling on both hands. Nine months earlier, he’d been diagnosed with bronchiolitis obliterans with organizing pneumonia (BOOP). At that time, he’d suffered from shortness of breath and pleural effusions and had undergone therapy with 40 mg q.d. of prednisone.
Upon Examination
We noted periungual telangiectases and erythematous papules over the dorsum of the metacarpal, proximal and distal interphalangeal joints of both hands. The patient had erythema and cracking on the palms of both his hands, as well as nail dystrophy of his index fingers and thumbs. He had violaceous macular lesions over the extensor surfaces of his lower extremities. He also had scattered papules on his upper body. Motor strength was 4/5 for the proximal muscle groups of the upper and lower extremities bilaterally and 5/5 for the distal muscle groups of the lower and upper extremities. Testing revealed a normal complete blood count, but elevated liver enzymes. His enzymes, including AST and ALT, were elevated at 57 U/L and 61 U/L, respectively. Lactate dehydrogenase, creatinine kinase and aldolase were elevated at 347, 762 and 30.5, respectively. Anti-nuclear antibody (ANA) was 1:40 and anti-Jo-1 was significantly elevated. Skin biopsy from an erythematous papule on the man’s lower back showed perivascular lymphocytic infiltrate, increased dermal edema with positive colloidal iron for mucin.
Considering the Diagnosis
After evaluating the patient history, laboratory data and skin biopsy results, we concluded the patient was suffering from dermatomyositis (DM). To rule out underlying malignancy, we ordered a colonoscopy, radiographs of the chest and a prostate specific antigen test. All were negative. Dermatomyositis is an inflammatory disease with characteristic skin manifestations and muscular weakness. The disease may be categorized as adult, idiopathic, juvenile or amyopathic dermatomyositis (dermatomyositis sine myositis), as well as that associated with malignancy.
Cutaneous Findings
These include periungual telangiectases, a periorbital erythematous/violaceous (heliotrope) eruption, reddish to violaceous, scaly papules or plaques on extensor surfaces (Gottron’s sign). Flat-topped violaceous papules over the metacarpal and interphalangeal joints are called Gottron’s papules and are thought to be pathognomonic of DM. Erythematous, poikilodermatous macules in a shawl distribution over the shoulders, arms and upper back are also common. Other less common skin findings include mechanic’s hands (fissured, scaly, hyperkeratotic and hyperpigmented hands), facial swelling and acquired ichthyosis. Muscle inflammation presents as proximal weakness and an associated elevation of muscle-derived enzymes including LDH, ALT and AST, and especially creatinine kinase and aldolase. Up to one-third of patients with primary idiopathic DM initially present only with skin changes, but characteristic systemic weakness usually develops within 2 years from the onset of the rash. Also, adults with DM are more likely to develop an internal malignancy, but not children who have the disease. Positive ANAs occur in 60% to 80% of cases, with 35% to 40% having myositis-specific antibodies. In addition, the incidence of interstitial lung disease and polymyositis in patients with DM may be as high as 45%. The three major types of interstitial lung disease associated with DM and polymyositis are interstitial pneumonia, diffuse alveolar damage and BOOP. The pathogenesis of the disease most likely involves immunologic factors; however, genetic and environmental issues may also contribute. Immunosuppressive agents are successful in treating most patients, although the disease still causes significant morbidity.
All authors are with the Georgetown University Medical Center in Washington, D.C.
A 67-year-old Caucasian man presented to our clinic with a 4-month history of erythema and scaling on both hands. Nine months earlier, he’d been diagnosed with bronchiolitis obliterans with organizing pneumonia (BOOP). At that time, he’d suffered from shortness of breath and pleural effusions and had undergone therapy with 40 mg q.d. of prednisone.
Upon Examination
We noted periungual telangiectases and erythematous papules over the dorsum of the metacarpal, proximal and distal interphalangeal joints of both hands. The patient had erythema and cracking on the palms of both his hands, as well as nail dystrophy of his index fingers and thumbs. He had violaceous macular lesions over the extensor surfaces of his lower extremities. He also had scattered papules on his upper body. Motor strength was 4/5 for the proximal muscle groups of the upper and lower extremities bilaterally and 5/5 for the distal muscle groups of the lower and upper extremities. Testing revealed a normal complete blood count, but elevated liver enzymes. His enzymes, including AST and ALT, were elevated at 57 U/L and 61 U/L, respectively. Lactate dehydrogenase, creatinine kinase and aldolase were elevated at 347, 762 and 30.5, respectively. Anti-nuclear antibody (ANA) was 1:40 and anti-Jo-1 was significantly elevated. Skin biopsy from an erythematous papule on the man’s lower back showed perivascular lymphocytic infiltrate, increased dermal edema with positive colloidal iron for mucin.
Considering the Diagnosis
After evaluating the patient history, laboratory data and skin biopsy results, we concluded the patient was suffering from dermatomyositis (DM). To rule out underlying malignancy, we ordered a colonoscopy, radiographs of the chest and a prostate specific antigen test. All were negative. Dermatomyositis is an inflammatory disease with characteristic skin manifestations and muscular weakness. The disease may be categorized as adult, idiopathic, juvenile or amyopathic dermatomyositis (dermatomyositis sine myositis), as well as that associated with malignancy.
Cutaneous Findings
These include periungual telangiectases, a periorbital erythematous/violaceous (heliotrope) eruption, reddish to violaceous, scaly papules or plaques on extensor surfaces (Gottron’s sign). Flat-topped violaceous papules over the metacarpal and interphalangeal joints are called Gottron’s papules and are thought to be pathognomonic of DM. Erythematous, poikilodermatous macules in a shawl distribution over the shoulders, arms and upper back are also common. Other less common skin findings include mechanic’s hands (fissured, scaly, hyperkeratotic and hyperpigmented hands), facial swelling and acquired ichthyosis. Muscle inflammation presents as proximal weakness and an associated elevation of muscle-derived enzymes including LDH, ALT and AST, and especially creatinine kinase and aldolase. Up to one-third of patients with primary idiopathic DM initially present only with skin changes, but characteristic systemic weakness usually develops within 2 years from the onset of the rash. Also, adults with DM are more likely to develop an internal malignancy, but not children who have the disease. Positive ANAs occur in 60% to 80% of cases, with 35% to 40% having myositis-specific antibodies. In addition, the incidence of interstitial lung disease and polymyositis in patients with DM may be as high as 45%. The three major types of interstitial lung disease associated with DM and polymyositis are interstitial pneumonia, diffuse alveolar damage and BOOP. The pathogenesis of the disease most likely involves immunologic factors; however, genetic and environmental issues may also contribute. Immunosuppressive agents are successful in treating most patients, although the disease still causes significant morbidity.
All authors are with the Georgetown University Medical Center in Washington, D.C.
A 67-year-old Caucasian man presented to our clinic with a 4-month history of erythema and scaling on both hands. Nine months earlier, he’d been diagnosed with bronchiolitis obliterans with organizing pneumonia (BOOP). At that time, he’d suffered from shortness of breath and pleural effusions and had undergone therapy with 40 mg q.d. of prednisone.
Upon Examination
We noted periungual telangiectases and erythematous papules over the dorsum of the metacarpal, proximal and distal interphalangeal joints of both hands. The patient had erythema and cracking on the palms of both his hands, as well as nail dystrophy of his index fingers and thumbs. He had violaceous macular lesions over the extensor surfaces of his lower extremities. He also had scattered papules on his upper body. Motor strength was 4/5 for the proximal muscle groups of the upper and lower extremities bilaterally and 5/5 for the distal muscle groups of the lower and upper extremities. Testing revealed a normal complete blood count, but elevated liver enzymes. His enzymes, including AST and ALT, were elevated at 57 U/L and 61 U/L, respectively. Lactate dehydrogenase, creatinine kinase and aldolase were elevated at 347, 762 and 30.5, respectively. Anti-nuclear antibody (ANA) was 1:40 and anti-Jo-1 was significantly elevated. Skin biopsy from an erythematous papule on the man’s lower back showed perivascular lymphocytic infiltrate, increased dermal edema with positive colloidal iron for mucin.
Considering the Diagnosis
After evaluating the patient history, laboratory data and skin biopsy results, we concluded the patient was suffering from dermatomyositis (DM). To rule out underlying malignancy, we ordered a colonoscopy, radiographs of the chest and a prostate specific antigen test. All were negative. Dermatomyositis is an inflammatory disease with characteristic skin manifestations and muscular weakness. The disease may be categorized as adult, idiopathic, juvenile or amyopathic dermatomyositis (dermatomyositis sine myositis), as well as that associated with malignancy.
Cutaneous Findings
These include periungual telangiectases, a periorbital erythematous/violaceous (heliotrope) eruption, reddish to violaceous, scaly papules or plaques on extensor surfaces (Gottron’s sign). Flat-topped violaceous papules over the metacarpal and interphalangeal joints are called Gottron’s papules and are thought to be pathognomonic of DM. Erythematous, poikilodermatous macules in a shawl distribution over the shoulders, arms and upper back are also common. Other less common skin findings include mechanic’s hands (fissured, scaly, hyperkeratotic and hyperpigmented hands), facial swelling and acquired ichthyosis. Muscle inflammation presents as proximal weakness and an associated elevation of muscle-derived enzymes including LDH, ALT and AST, and especially creatinine kinase and aldolase. Up to one-third of patients with primary idiopathic DM initially present only with skin changes, but characteristic systemic weakness usually develops within 2 years from the onset of the rash. Also, adults with DM are more likely to develop an internal malignancy, but not children who have the disease. Positive ANAs occur in 60% to 80% of cases, with 35% to 40% having myositis-specific antibodies. In addition, the incidence of interstitial lung disease and polymyositis in patients with DM may be as high as 45%. The three major types of interstitial lung disease associated with DM and polymyositis are interstitial pneumonia, diffuse alveolar damage and BOOP. The pathogenesis of the disease most likely involves immunologic factors; however, genetic and environmental issues may also contribute. Immunosuppressive agents are successful in treating most patients, although the disease still causes significant morbidity.
All authors are with the Georgetown University Medical Center in Washington, D.C.
