Churg-Strauss syndrome: a case report

Area of Science:

• Rheumatology
• Immunology
• Dermatology

Background:

• Churg-Strauss syndrome (CSS), an allergic granulomatous angiitis, is a rare vasculitis affecting small and medium vessels.
• Diagnosis requires meeting at least four of six American College of Rheumatology criteria, including asthma, eosinophilia, sinusitis, pulmonary infiltrates, vasculitis with extravascular eosinophils, and neuropathy.
• Perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) are often associated with CSS.

Observation:

• An 80-year-old female with a long history of asthma and persistent peripheral eosinophilia presented with new-onset skin lesions.
• The skin manifestations included vesicles, vesiculopustules, purpuric macules, papules, and petechiae.
• Further investigations confirmed pulmonary infiltrates, paranasal sinusitis, extravascular eosinophils in skin biopsy, and polyneuropathy.

Findings:

• The presence of skin changes, alongside pre-existing asthma and eosinophilia, raised suspicion for CSS.
• Histopathological examination of skin tissue revealed extravascular eosinophils, a key diagnostic feature.
• The patient's presentation met the diagnostic criteria for CSS.

Implications:

• This case highlights the importance of considering CSS in patients with asthma and eosinophilia, especially when cutaneous manifestations arise.
• Early recognition and treatment, including corticosteroids and cyclophosphamide, are crucial for managing CSS.
• The resolution of skin lesions suggests the efficacy of the initiated treatment regimen.