Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Bulbar, respiratory or rapidly progressive lung disease
Dysphagia, aspiration, weak cough, orthopnoea, falling respiratory strength, hypoxia or rapidly worsening breathlessness can reflect bulbar myositis, respiratory-muscle failure or aggressive interstitial lung disease.
Action: Admit for swallowing, gas exchange, seated and supine respiratory testing and urgent rheumatology, respiratory and critical-care input; restrict unsafe oral intake and treat infection and immune disease in parallel.
Synopsis
Recognise dermatomyositis across skin tones, detect clinically amyopathic and organ-threatening phenotypes, direct muscle, lung and malignancy assessment, and prevent scarring, calcinosis and treatment harm.
Gottron papules are violaceous or erythematous papules over knuckles; Gottron sign is flat change over extensor joints. Heliotrope eyelid colour and oedema are also characteristic.
Photosensitive shawl, V-neck and lateral-thigh holster eruptions, scalp inflammation, ragged cuticles and abnormal nailfold capillaries add a coherent skin phenotype.
In brown or black skin, inflammation may look purple-brown, grey or hyperpigmented and scale or oedema may be more visible than red colour.
Key red flags
Skin ulceration, palmar papules, anti-MDA5 phenotype, new dyspnoea, dysphagia, objective proximal weakness, myocarditis symptoms, constitutional decline or malignancy warning features requires expedited multidisciplinary assessment even when creatine kinase is normal.
Ulcerative MDA5 pattern
Painful ulcers over Gottron sites or nailfolds and palmar papules raise rapidly progressive ILD concern even without weakness.
Investigation priorities
01
Functional and manual muscle assessmentFirst step
Prove weakness, map distribution and establish an outcome baseline.
Management branches
First diagnostic sequenceUse skin to trigger whole-organ mapping
Gottron, heliotrope, photodistributed or nailfold features suggest dermatomyositis.
Photograph skin in neutral light with consent and examine scalp, eyelids, knuckles, nails, hands and thighs, then measure proximal, neck and respiratory function and ask about swallow.
Request muscle enzymes, renal and urine tests and phenotype-directed antibodies, adding skin biopsy, muscle MRI, EMG or muscle biopsy when the diagnosis remains uncertain.
First-line skin treatmentProtect from light and suppress local activity
Cutaneous disease is limited and no organ-threatening feature requires immediate systemic rescue.
Key medicines
Site-appropriate topical corticosteroidApply a thin layer once daily to active nonfacial skin or scalp for a defined two-to-four-week course, then review before reducing or continuing intermittently.
Hydroxychloroquine for skin-predominant diseaseTake 200–400 mg by mouth once daily with food without exceeding 5 mg/kg actual body weight daily, under specialist response and retinal review.
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.