Make the Diagnosis: More Than A Rash

Presentation

Case Findings: A 51-year-old woman visited her primary care doctor complaining of a rash, pruritus, myalgias, and muscle weakness that made normally easy tasks like standing from a seated position difficult. The rash was an erythematous blanching patch, spread across her back and shoulders like a shawl and across her chest in a V shape. She had also noticed that her fingernail folds were red and swollen.

What is your diagnosis?

Learnings

Dermatomyositis is a multisystem autoimmune connective tissue disease that is most often characterized by a symmetric proximal extensor inflammatory myopathy, a characteristic violaceous cutaneous eruption, and pathogenic circulating autoantibodies. Dermatomyositis demonstrates a bimodal incidence, with the adult form most commonly seen in individuals aged 45-60, and the juvenile form found in children aged 10-15 years. A 2:1 female-to-male incidence ratio exists in adults.

While the etiology remains unclear, some evidence suggests that genetically susceptible individuals with certain HLA types mount aberrant cellular and humoral responses after exposure to malignancy, infection, or drug ingestion.

Clinical features of dermatomyositis can be categorized into cutaneous and systemic manifestations. Typical findings include a heliotrope rash, atrophic dermal papules of dermatomyositis (ADPDM; previously Gottron's papules), shawl sign, holster sign, photosensitivity, flagellate erythema, poikiloderma, calcinosis cutis and nail fold changes. Pruritus is also common.

Systemic manifestations of dermatomyositis include fatigue, malaise, myalgias, and the following:

• Musculoskeletal – Proximal extensor muscle group inflammation that leads to muscle pain and weakness. Patients may have difficulty getting up from a sitting position or combing their hair.
• • Gastrointestinal – Dysphagia can be seen due to esophageal muscle involvement.

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Make the Diagnosis: More Than A Rash