A Rare Case of Eosinophilic Granulomatosis with Polyangiitis Associated with Cryoglobulinemia Presenting with a Bullous Skin Eruption of the Lower Limbs

Abstract EN

Background.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody- (ANCA-) associated small vessel vasculitis with multisystem involvement.

It is characterized with asthma, eosinophilia, and renal and peripheral nervous system involvement.

However, EGPA presenting with bullous skin eruption is an uncommon dermatological manifestation.

We report a rare case of EGPA overlapped with mixed essential cryoglobulinemia presenting with a bullous skin eruption.

Case Presentation.

A 49-year-old female presented with bilateral lower limb erythematous bullous rash with bilateral lower limb numbness.

She had bilateral ankle edema with frothyuria and a recent onset wheeze.

Blood investigations revealed a marked peripheral eosinophilia with positive P-ANCA.

Skin biopsy was suggestive of leukocytoclastic vasculitis.

She also had positive cryoglobulins with a high rheumatoid factor titre.

The patient was diagnosed of having EGPA with overlapping mixed essential cryoglobulinemia.

Her skin eruptions and systemic manifestations improved with prednisolone and cyclophosphamide therapy.

Conclusion.

EGPA can rarely present with a bullous skin eruption and may rarely associate with secondary cryoglobulinemia.

Early recognition of these rare manifestations and prompt treatment would prevent further complications and death.