What is the treatment approach for Rowell syndrome?

Rowell Syndrome Treatment

For Rowell syndrome, initiate systemic corticosteroids (methylprednisolone 0.5-1 mg/kg/day) combined with hydroxychloroquine 400 mg/day as first-line therapy, and immediately discontinue any suspected triggering medications, particularly proton pump inhibitors. 1, 2, 3

Immediate Management Steps

Identify and Remove Triggers

• Discontinue any recently started medications, especially proton pump inhibitors (omeprazole, esomeprazole), terbinafine, antibiotics (norfloxacin), or anticonvulsants (sodium valproate), as these are the most commonly implicated drug triggers 1, 3
• Replace proton pump inhibitors with H2-receptor antagonists (famotidine 20 mg/day) if gastroprotection is needed 1
• Assess for recent herpes simplex infection or significant sun exposure as potential precipitants 4

First-Line Pharmacologic Treatment

• Start oral methylprednisolone 0.5-1 mg/kg/day as the cornerstone of acute management 2, 3, 5
• Add hydroxychloroquine 400 mg/day concurrently for its immunomodulatory effects and long-term disease control 2, 5, 4
• Apply topical corticosteroids (fluticasone propionate 0.05% cream once daily) to affected areas 3, 4
• Consider topical calcineurin inhibitors as adjunctive therapy for facial or mucosal involvement 4

Management of Refractory Disease

Escalation for Rapid Progression

• If cutaneous involvement progresses rapidly despite oral corticosteroids, administer pulse methylprednisolone 500 mg/day intravenously for three consecutive days 2
• Upon failure of pulse steroids, initiate oral cyclosporine 3.5 mg/kg/day, which has demonstrated excellent and complete clinical response within 4 weeks in steroid-resistant cases 2
• Gradually taper systemic corticosteroids once cyclosporine achieves clinical control 2

Alternative Immunosuppressive Options

• Add azathioprine 50 mg/day as a steroid-sparing agent in patients requiring prolonged immunosuppression 3
• Consider dapsone, methotrexate, or retinoids for anti-malarial resistant disease, though evidence is limited 4

Supportive Care

Gastroprotection and Monitoring

• Provide gastroprotection with H2-receptor antagonists (not proton pump inhibitors) when using systemic corticosteroids 1, 3
• Add potassium supplementation (KCl 1 g/day) during prolonged corticosteroid therapy 3

Expected Timeline

• Clinical improvement typically occurs within 1 month of appropriate therapy with drug discontinuation and hydroxychloroquine dose optimization 1
• Complete response to cyclosporine can be expected by week 4 in steroid-refractory cases 2

Critical Diagnostic Confirmation

Before initiating treatment, confirm the diagnosis requires all three major criteria: lupus erythematosus (any subtype), erythema multiforme-like lesions, and speckled pattern antinuclear antibodies, plus at least one minor criterion (chilblains, anti-Ro/SSA or anti-La/SSB antibodies, or positive rheumatoid factor) 2, 3, 5

Common Pitfalls

• Do not continue proton pump inhibitors during treatment, as they are a major cause of drug-induced subacute cutaneous lupus erythematosus and can perpetuate Rowell syndrome 1, 3
• Do not delay escalation to cyclosporine in rapidly progressive cases unresponsive to pulse steroids, as this agent provides rapid and dramatic effectiveness 2
• Do not use cyclosporine monotherapy initially; it should be reserved for steroid-refractory cases rather than first-line treatment 2
• The latency between drug initiation and syndrome onset can exceed 1 year, so maintain high suspicion for drug causation even with prolonged medication use 1