Paraneoplastic dermatomyositis: a study of 12 cases.
OBJECTIVE
Adult dermatomyositis presents as a paraneoplastic syndrome in up to 25% of cases, but no clinical, histologic, or laboratory markers completely specific for paraneoplastic disease in dermatomyositis have been identified to date. Furthermore, studies on adult dermatomyositis do not usually report the frequency of cutaneous features of dermatomyositis in patients with associated cancer. Our aim was to review the characteristics of paraneoplastic dermatomyositis in patients seen at our hospital.


METHODS
We studied 12 cases of paraneoplastic dermatomyositis and recorded patient age and sex, associated cancer, time between onset of dermatomyositis and cancer, emergent cutaneous manifestations, muscle involvement, dysphagia, lung disease, and levels of creatine phosphokinase and circulating autoantibodies.


RESULTS
The mean age of the patients was 61 years and the 2 most common malignancies were ovarian cancer and bladder cancer. The mean time between the diagnosis of cancer and dermatomyositis was 7 months and in most cases, the cancer was diagnosed first. Seven patients had amyopathic dermatomyositis. The most common cutaneous signs were a violaceous photodistributed rash sparing the interscapular area and a heliotrope rash, followed by Gottron papules and cuticle involvement. Superficial cutaneous necrosis was observed in 3 cases. Myositis-specific autoantibodies were not detected in any of the 6 patients who underwent this test.


CONCLUSIONS
Paraneoplastic dermatomyositis is often amyopathic. There are no specific cutaneous markers for malignancy in dermatomyositis. Myositis-specific antibodies are not associated with paraneoplastic dermatomyositis.
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España. Electronic address: celiareq@hotmail.com.
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Requena
Servicio de Dermatología, Hospital de Manises, Valencia, España.
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Alfaro
Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, España.
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Traves
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
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Nagore
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
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Llombart
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
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Serra
Servicio de Dermatología, Hospital de Manises, Valencia, España.
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Martorell
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España.
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Guillén
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España; Departamento de Patología Médico-Quirúrgica, Universidad Católica de Valencia, San Vicente Mártir, Valencia, España.
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Sanmartín
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000150:complications / Q000175:diagnosis; D012189:Retrospective Studies
Amyopathic dermatomyositis; Cancer; Cáncer; Dermatomiositis amiopática; Heliotrope rash; Necrosis; Paraneoplastic; Paraneoplásica; Rash heliotropo