churg - strauss syndrome ( css ) so called allergic granulomatosis is a rare primary , non - inheritable , non - transmissible systemic disease , which typically occurs in patients with a history of bronchial asthma . peripheral eosinophilia and medium and small vessel autoimmune necrotizing vasculitis , involving multiple organs are the main features of the disease . css can be diagnosed by the presence of any four or more of the six criteria , including asthma , eosinophilia greater than 10% , paranasal sinusitis , pulmonary infiltration , histologically confirmed vasculitis and neuropathy . a fifty - year - old female presented with a one month history of progressive dyspnea , productive cough , pain of elbows and knees , and 40c fever despite antibiotic treatment . she had been diagnosed with bronchial asthma over 25 years before admission and oral and depot glucocorticosteroids as a long - term therapy was applied . physical examination revealed tachypnea , wheezes , rhonchi and wet cracles on auscultation , tachycardia , skin nodules , urticarial rash and necrotic bullae all over the body ( table 1 , figure 1 ) . chest x - ray showed transient , patchy , nonsegmental areas of consolidation , with predilection for lower zones with the area of consolidation in lower left zone ( table 1 , figure 2a ) . transbronchial lung biopsy revealed granulomas , formed by eosynophils with central necrosis , macrophages and giant cells ( table 1 , figure 2b ) . a - x - ray of the chest : transient , patchy , nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone ; b - transbronchial lung biopsy : granulomas , formed by eosynophils with central necrosis , macro phages and giant cells . patient was diagnosed with churg strauss syndrome and initial treatment with prednisone was introduced at a dose of 60 mg per day , i.e. 1 mg / kg / day . after four days of treatment , after 18 months of treatment , the patient reported no signs or symptoms of the disease . patient continues oral corticosteroid therapy at a dose of 10 mg of prednisone per day . idiopathic primary systemic vasculitides are defined , according to the chapel hill consensus conference , on the basis of the size of vessels involved , clinical symptoms , and histopathological features . according to the american college of rheumatology ( acr ) , classification criteria of the css are : small vessel vasculitis along with wegener 's granulomatosis ( wg ) , microscopic polyangiitis ( mpa ) , and idiopathic necrotizing crescentic glomerulonephritis ncgn . although , css can affect any organ system in the body , lung blood vessels involvement is one of the main features of the disease . asthma , peripheral eosinophilia , peripheral neuropathy , pulmonary infiltrates , and paranasal sinus abnormalities remain the major classification criteria of the disease . our patient had been diagnosed with bronchial asthma over 25 years before admission . skin lesions are the most common extra - pulmonary findings in the course of the disease . the main types of skin lesions reported include erythematous maculopapules resembling erythema multiform , hemorrhagic lesions ranging from petechiae to extensive ecchymosis , often associated with wheals , necrosis and ulceration , and cutaneous and subcutaneous nodules that are usually deep - seated and tender with a predilection for the scalp and temple region . others report livedo reticularis , papulovesicles , non - pitting periorbital edema , and nail - fold infarcts [ 4 - 8 ] . skin nodules urticarial rash and necrotic bullae all over the body were found in our patient . regarding clinical features , our patient complained of progressive dyspnea , productive cough , pain of elbows and knees , and 40c fever , despite the antibiotic and steroid treatment . we also found radio - logic changes , such as pulmonary infiltrates detected by x - ray and computed tomography , and other manifestations including , mononeuritis multiplex , eosino philic endomyocarditis , with no renal involvement documented . these are consistent with main clinical findings describing the disease [ 1,3 - 5 ] . we did not find positive antineutrophil cytoplasmic antibodies ( anca ) in our patient . according to reid et al   and della rosa et al   positive anca may be found in 39 to 59% of patients with css . sable - fourtassou et al   found the predominance of glomerulonephritis and peripheral neuropathy among positive anca patients , and more frequent cardiac involvement , and fever , among anca negative patients . based on that , these authors hypothesized that anca positivity may reect an active vasculitic process , while anca negativity may be more frequently linked to another clinical phenotype that could correspond to different pathogenic mechanisms which are less often associated with anca . consistently with their hypothesis , cardiac involvement and no gromerulonphritis characterized our case , with the exception for peripheral neuropathy presented . according to sable - fourtassou et al   a higher frequency of vasculitis in biopsy specimens from the anca - positive group and a close association of renal involvement with anca positivity support a pathogenic role for anca . kallenberg et al   focusing on anca directed to proteinase-3 ( pr3-anca ) or myeloperoxidase ( mpo - anca ) summarized that anca alone are not sufficient , as based on clinical and experimental data , and other , probably exogenous factors , seem necessary for disease induction and ( re)activation . in particular , silica , the carriage of staphylococcus aureus , and various genetic factors may underline susceptibility to the disease . we did not find any bacterial source of infection or any genetic factors in our patient . concerning treatment , our patient received a conventional prednisone therapy , resulting in early regression of clinical features . further dose of the drug was titrated according to the level of blood eosinophils , with no signs or symptoms of the disease during a follow - up of 18 months of therapy . despite novel therapies being introduced , swietlik and doboszynska   suggest the use of inhaled corticosteroids as a recurrence preventing approach and this may be a further step to be taken in our patient . lamprecht et al   in their most recent publication summarize that anti - cd20 therapy and tnf - alpha - antagonists may represent strategies for refractory disease , but evidence from controlled trials is still lacking .
a fifty - year - old female presented with a one month history of progressive dyspnea , productive cough , pain of elbows and knees , and 40c fever despite antibiotic treatment . 
 she has been diagnosed of bronchial asthma over 25 years before admission and oral and depot glucocorticosteroids as a long - term therapy was applied . 
 recently , an attempt of inhaled corticosteroids and laba treatment was introduced with no success . 
 four years before admission she also developed peripheral neuropathy . 
 physical examination revealed tachypnea , wheezes , rhonchi and wet cracles on auscultation , tachycardia , skin nodules , urticarial rash and necrotic bullae all over the body . 
 chest x - ray showed transient , patchy , nonsegmental areas of consolidation with predilection for lower zones with the area of consolidation in lower left zone . 
 obstruction was found on spirometry . 
 tachycardia on ecg and myocardial fluid on echo were also detected . 
 lab exams revealed elevated crp , wbc , eosinophils , and ige levels . 
 ana and anca antibodies were not found . 
 patient was diagnosed of churg strauss syndrome and initial treatment of prednisone was introduced . 
 after four days of treatment , temperature normalized , and dyspnea diminished . 
 after one month of therapy skin lesions regressed . 
 after 18 months of the treatment patient reports no signs , nor symptoms of the disease . 
 patient continues oral corticosteroid therapy .