Eosinophilic Granulomatosis With Polyangiitis With Extensive Cutaneous Involvement

Area of Science:

• Rheumatology
• Dermatology
• Internal Medicine

Background:

• Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis characterized by asthma, eosinophilia, and systemic organ involvement.
• EGPA diagnosis can be challenging, particularly in its early stages or with atypical presentations.

Observation:

• A 68-year-old female presented with extensive hemorrhagic bullous lesions, tissue loss, and necrosis on elbows, hands, feet, and knees.
• The patient was diagnosed with antineutrophil cytoplasmic antibody-positive EGPA with cutaneous, pulmonary, renal, intestinal, and nervous system involvement.
• Initial treatment with corticosteroids and intravenous immunoglobulin led to infectious complications from multidrug-resistant bacteria.

Findings:

• Despite initial treatment, the patient had persistent disabling dysesthesias and extensive skin ulcers.
• Successful management involved resolving infections, followed by rituximab therapy and surgical wound repair (debridement and grafting).
• The patient achieved complete wound healing and improved quality of life after multidisciplinary treatment.

Implications:

• This case underscores the complexity of managing severe, multisystem EGPA, balancing immunosuppression with infection risk.
• Aggressive treatment, including novel therapies and surgical interventions, can lead to significant recovery in severe EGPA cases.
• Optimizing patient quality of life requires a comprehensive approach addressing both systemic disease activity and its cutaneous manifestations.