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Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a condition characterized by asthma, high levels of eosinophils and vasculitis. It is characterized during its prodromal phase by an asthma of increasing severity. Vasculitis affects the lungs, the heart, the skin and the peripheral nerves. Skin involvement is common but may not be very suggestive.
The authors report the case of a 39-year-old man, with a medical history of asthma and sinusitis, who consulted in the ER for abdominal pain and partial occlusion syndrome. Diagnostic laparoscopy showed images of digestive vasculitis. Skin biopsy of an axillary subcutaneous mass which had lipomatous aspect was compatible with vasculitis associated with a perivascular infiltrate rich in eosinophils. Biology showed a persistent hyper eosinophilia in the blood. Antineutrophil cytoplasmic antibodies were negative. There was no evidence of pulmonary infiltrate or sinus opacity. The other cardiac, neurological and renal explorations were normal. The diagnosis of EGPA was suspected based on clinical and biological arguments. It was confirmed by skin biopsy of an atypical axillary mass. Treatment with corticosteroid therapy and immunosuppressants resulted in spectacular clinical improvement with a 3-year follow-up.
The cutaneous manifestations can have an essential contribution for the diagnosis of vasculitis. Early recognition of these lesions is essential for proper treatment and prevention of serious visceral complications.
Our observation is unique due to the atypical cutaneous manifestation which contributed to the early positive diagnosis of EGPA.
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