[Churg-Strauss syndrome presenting with cutaneous vasculitis]. / Síndrome de Churg-Strauss - Vasculite cutânea como forma de apresentação clínica.

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium sized vessels vasculitis. Vasculitis commonly affects lung, heart, skin and peripheral nerves. The authors report a case of a 47-year-old woman that was admitted with a 1-week history of painful purpuric lesions affecting extensor surfaces of lower extremities. She had a 7 years-long medical history of asthma, paranasal sinusitis and allergic rhinitis. Laboratorial tests revealed leucocytosis, peripheral blood eosinophilia and elevated inflammatory parameters. Antineutrophil cytoplasmic antibodies were negative. Skin biopsy was consistent with necrotizing vasculitis with perivascular eosinophil-rich infiltrate. Electromyogram and nerve conduction studies showed asymmetrical peripheral polyneuropathy. Churg-Strauss syndrome diagnosis was supported by clinical and histopathological findings. High-dose oral steroid therapy led to a dramatic clinical improvement. Skin manifestations can be the presenting feature of Churg-Strauss syndrome. Early recognition is essential for appropriate treatment and prevention of irreversible organ damage.

[Acute generalized exanthematous pustulosis associated with diltiazem]. / Pustulose generalizada exantemática aguda associada ao diltiazem.

Acute generalized exanthematous pustulosis (AGEP) usually presents with the acute appearance of oedematous and erythematous lesions, on which multiple sterile pustules appear, associated with fever. Almost 90% of cases are associated with drugs, with antibiotics (penicillins and macrolides) being the most frequent causative agents. We describe a 36-year-old female patient, which started diltiazem 120mg/day for hypertension. After 6 days of therapy, multiple erythematous and oedematous lesions appeared, with associated multiple small non-follicular pustules. Oral corticosteroids were started, with progressive and complete improvement. Patch-tests were performed, which revealed positivity for diltiazem. Although a rare entity, AGEP must be considered in cases of acute eruptions with disseminated pustules and fever. The use of patch tests in this disease may be useful as positive reactions are frequent.