Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a mar...Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a marked peripheral blood eosinophilia, but the pathogenesis remains unknown. Observations: A retrospective review was performed in 9 cases of CSS in whom cutaneous findings were present as an initial manifestation. All 9 patients had purpura and petechiae as well as severe pain and paresthesias of the lower extremities. Four patients (44%) used leukotriene receptor antagonists to treat their asthma, and 3 (75%) of them developed CSS within 3 months. Five patients (56%) were positive for perinuclear antineutrophil cytoplasmic antibodies before therapy, but in all 5 the levels of perinuclear antineutrophil cytoplasmic antibody normalized. Serum IgE levels were elevated in all patients before treatment but decreased after treatment. Histologically, all patients demonstrated leukocytoclastic vasculitis and eosinophilic infiltration. Eight biopsy specimens (73%) revealed marked eosinophilia around the nerve fibers in the dermis. Palisading granulomas in association with vessel-based changes were present in 4 (36%) of 11 biopsy specimens. Conclusions: These characteristic cutaneous clinical patterns that are consistent with the presence of mononeuropathy multiplexes in the lower extremities may help physicians establish an earlier diagnosis. Both eosinophils and IgE, as well as perinuclear antineutrophil cytoplasmic antibodies to some degree, likely participate in skin lesion development in CSS. Furthermore, there appears to be a correlation between treatment with leukotriene receptor antagonists and the onset of CSS in some cases.展开更多
Microscopic polyangiitis is a systemic small vessel vasculitis,which often has cutaneous and musculoskeletal features.Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody(ANC...Microscopic polyangiitis is a systemic small vessel vasculitis,which often has cutaneous and musculoskeletal features.Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody(ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase(MPO)-ANCA.Titres of MPO-ANCA may reflect disease activity and play a pathogenic role.Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation.Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils,consistent with leukocytoclastic vasculitis,in the deep dermis to the subcutaneous fat tissue.The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms,such as arthralgias and myalgias.The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres.展开更多
文摘Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a marked peripheral blood eosinophilia, but the pathogenesis remains unknown. Observations: A retrospective review was performed in 9 cases of CSS in whom cutaneous findings were present as an initial manifestation. All 9 patients had purpura and petechiae as well as severe pain and paresthesias of the lower extremities. Four patients (44%) used leukotriene receptor antagonists to treat their asthma, and 3 (75%) of them developed CSS within 3 months. Five patients (56%) were positive for perinuclear antineutrophil cytoplasmic antibodies before therapy, but in all 5 the levels of perinuclear antineutrophil cytoplasmic antibody normalized. Serum IgE levels were elevated in all patients before treatment but decreased after treatment. Histologically, all patients demonstrated leukocytoclastic vasculitis and eosinophilic infiltration. Eight biopsy specimens (73%) revealed marked eosinophilia around the nerve fibers in the dermis. Palisading granulomas in association with vessel-based changes were present in 4 (36%) of 11 biopsy specimens. Conclusions: These characteristic cutaneous clinical patterns that are consistent with the presence of mononeuropathy multiplexes in the lower extremities may help physicians establish an earlier diagnosis. Both eosinophils and IgE, as well as perinuclear antineutrophil cytoplasmic antibodies to some degree, likely participate in skin lesion development in CSS. Furthermore, there appears to be a correlation between treatment with leukotriene receptor antagonists and the onset of CSS in some cases.
文摘Microscopic polyangiitis is a systemic small vessel vasculitis,which often has cutaneous and musculoskeletal features.Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody(ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase(MPO)-ANCA.Titres of MPO-ANCA may reflect disease activity and play a pathogenic role.Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation.Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils,consistent with leukocytoclastic vasculitis,in the deep dermis to the subcutaneous fat tissue.The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms,such as arthralgias and myalgias.The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres.
