What is Rowell Syndrome?
Rowell syndrome is a rare autoimmune disorder characterized by the simultaneous occurrence of lupus erythematosus (LE), erythema multiforme (EM)-like skin lesions, and specific immunological abnormalities including speckled pattern antinuclear antibodies. 1
Diagnostic Criteria
The diagnosis requires meeting all three major criteria plus at least one minor criterion 2, 3:
Major Criteria (All Required):
- Lupus erythematosus (systemic, discoid, or subacute cutaneous forms) 3
- Erythema multiforme-like skin lesions with target or annular patterns, often with hemorrhagic crusts 4
- Speckled pattern antinuclear antibodies (ANA) on indirect immunofluorescence 5
Minor Criteria (At Least One Required):
Clinical Presentation
The skin lesions typically appear as erythematous infiltrated plaques with annular or target patterns, predominantly affecting sun-exposed areas including the neck, chest, upper back, and shoulders 4. The lesions characteristically form hemorrhagic crusts and scales at the margins, while mucous membranes are usually spared 4.
Associated systemic symptoms include:
- Fatigability and myalgia 2
- Arthritis (lupus arthritis) 4
- Constitutional symptoms related to underlying lupus 1
Triggering Factors
Multiple precipitating factors have been identified 1:
Medications (most common drug-induced triggers):
Infections:
Environmental factors:
Laboratory Findings
Essential laboratory evaluation includes 1, 4:
- Positive speckled ANA (often >1:1280 titers) 4
- Anti-Ro/SSA antibodies (>200 U/mL) 4
- Anti-La/SSB antibodies (>200 U/mL) 4
- Rheumatoid factor (may be positive) 5
- Complete blood count with differential 1
- 24-hour urine protein (to assess for lupus nephritis) 4
Direct immunofluorescence from lesional skin typically shows granular C3 deposits at the dermo-epidermal junction 4.
Histopathological Features
Skin biopsy reveals overlapping features of both lupus erythematosus and erythema multiforme 4:
- Epidermal atrophy with vacuolar degeneration of the basal layer 4
- Sparse perivascular lymphocytic infiltrate in the dermis 4
- Single eosinophilic necrotic keratinocytes (characteristic of EM) 4
Treatment Approach
Systemic corticosteroids serve as first-line therapy, with hydroxychloroquine as the cornerstone of long-term management 1, 5:
Initial Management:
- Methylprednisolone 0.5 mg/kg/day for acute flares 2
- Hydroxychloroquine 200-400 mg/day for maintenance 4, 5
- Immediate discontinuation of suspected triggering medications 4
Additional Therapeutic Options:
- Azathioprine 50 mg/day for steroid-sparing immunosuppression 2
- Topical corticosteroids (fluticasone propionate 0.05% cream) 2
- Topical calcineurin inhibitors 3
- Rituximab for refractory cases (showing favorable outcomes) 1
Supportive Measures:
- Gastroprotection with H2-receptor antagonists (famotidine 20 mg/day) if PPIs must be avoided 4
- Potassium supplementation (1 g/day) when on systemic corticosteroids 2
- Treatment of superimposed infections (ceftriaxone 2 g/day for bacterial infections) 4
Important Clinical Pitfalls
The latency period between drug initiation and syndrome onset can exceed one year, making causality assessment challenging 4. This is particularly true for proton pump inhibitors and diuretics, which have longer latency periods compared to antifungals or chemotherapeutics 4.
Clinical improvement typically occurs within one month after discontinuing the offending agent and optimizing immunosuppressive therapy 4. However, the existence of Rowell syndrome as a distinct entity versus a coincidental overlap remains debated in the literature 4.
Sun protection is critical as sun-exposed areas are preferentially affected, and UV exposure can trigger or exacerbate lesions 3.