SHM Converge Scientific Abstract ePoster Gallery-Paraneoplastic Dermatomyositis Secondary to Gastrointestinal Stromal Tumor

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This case describes an 84-year-old man with hyperlipidemia who presented with six weeks of erythematous rash, proximal muscle weakness, and later dysphagia. His exam showed classic dermatomyositis findings, including a shawl-like rash, periorbital edema, cutaneous ulceration, Gottron’s papules, and periungual erythema, with symmetric proximal weakness in both upper and lower extremities. Laboratory workup showed markedly elevated creatine kinase, elevated ESR, and a high ANA titer, while anti-Jo-1, mitochondrial, and smooth muscle antibodies were negative.<br /><br />He was initially treated as an outpatient with steroid taper after skin biopsy confirmed dermatomyositis, but his weakness worsened and dysphagia developed, prompting hospital admission. In the hospital, he received pulse-dose steroids without improvement, followed by high-dose oral steroids. Because of persistent disease, IVIG was started on hospital day 7 and seemed to improve symptoms, leading to methotrexate maintenance therapy. CT and PET imaging then revealed a gastric mass, and biopsy confirmed a spindle cell gastrointestinal stromal tumor (GIST). Neoadjuvant imatinib was started on day 16.<br /><br />Despite treatment, the patient deteriorated on day 29 with septic shock from aspiration pneumonia, requiring intubation and vasopressors. He was unable to be weaned from the ventilator and transitioned to comfort care, dying on hospital day 47.<br /><br />The case highlights several important points: updated idiopathic inflammatory myopathy criteria may have allowed earlier dermatomyositis diagnosis; cutaneous ulceration may indicate a severe, treatment-resistant course; and delays in IVIG may worsen dysphagia and aspiration risk. It also emphasizes the need for malignancy screening in dermatomyositis, including rare paraneoplastic causes such as GIST, which may respond well to imatinib if localized.

Asset Subtitle

Daniel M. Camp

Meta Tag

Author List Daniel M. Camp, Frank Aguilar

Category Clinical Vignettes

Concept Dermatomyositis

Concept Paraneoplastic Syndrome

Concept Malignancy Screening

Concept Clinical Feature

Concept Diagnostic Approach

Distinguished Non-Finalist

Presenter Organization Northwestern University

Presenting Author Daniel M. Camp

Track Adult

Keywords

dermatomyositis

proximal muscle weakness

dysphagia

cutaneous ulceration

Gottron’s papules

elevated creatine kinase

intravenous immunoglobulin

gastrointestinal stromal tumor

paraneoplastic syndrome

imatinib

Dermatomyositis

Paraneoplastic Syndrome

Malignancy Screening

Clinical Feature

Diagnostic Approach