1. SATAN’S CURSE Saad Ghafoor MD (Associate), Saiprakash Venkateshiah MD, FCCP  Hyper Ig E syndrome or Job’s syndrome is a multisystem disorder that affects dentition, the skeleton, connective tissue and immune system.  This syndrome is characterized by recurrent sino pulmonary infections, cold abcesses, dermatitis and elevated IgE levels.  Neutrophils from affected patients show intermittent chemotactic defects, including deficient or absent IF gamma production, deficient IL-18 or defects in T cell signaling, resulting in a decrease neutrophil influx in areas of infection.  Affects men and women equally from diverse ethnic backgrounds.  Patients have characteristic facial features “ frontal Bossing” broad nose, prominent lower lip. Osteopenia due to cytokine mediated bone resorption.  Serum IgE levels range from 2000-50,000 IU/ml.  Patients are at an increased risk for lymphoma.  Staphylococcal pneumonias with pneumatocele strongly suggest diagnosis of HIE syndrome.  Inherited in sporadic or autosomal dominant pattern.  No single clinical or laboratory test exists for the diagnosis. It is a constellation of clinical signs, symptoms and laboratory studies to aid in the diagnosis of this rare disease. A 35 year old African American male was admitted with a four week history of cough with productive white colored sputum. He also reported fever, night sweats and 21 pound weight loss over the past month. His past medical history was significant for eczema since childhood and recurrent pneumonias for the last six years. He had 2 pack year history of smoking but quit two months prior to this hospital admission. He worked for a painting company where he has been exposed to fumes for the past year. PHYSICAL EXAM: He had a prominent forehead, broad nasal tip and thick tongue. Bronchial breath sounds with crackles heard in lower third of the right chest, along with decreased breath sounds at left base and scattered expiratory wheeze bilaterally. He had diffuse rash with areas of hyper and hypo pigmentation with scaling lichenification. Rest of the exam was unremarkable. HOSPITAL COURSE He was started on ceftriaxone and levofloxacin for a diagnosis of community acquired pnemonia. WBC count peaked at 20,000 with a maximum temperature of 101.3 F. Cefriaxone was switched to piperacillin-tazobactam. Blood cultures, AFB x 3 were negative. Bronchoscopy was done with BAL only growing rare Candida, but negative for AFB, bacteria and viruses. Transbronchial biopsies showed interstitial thickening, fibrinous debris along with areas of normal lung parenchyma. No organisms, granulomas or lymphoid aggregates were identified. Antibiotics were changed to vancomycin alone. He was discharged on a 7 day course of bactrim with a downward trending leukocyte count. WBC : 14,200/uL with a left shift and 8% bands Albumin: 2.0g/dL, Alpha-1 antitrypsin : 256mg/dL(83-199), ACE level 58U/L(9-67) IgA: 492(63-378) and IgE 12728 U/ml (1.3-165). Rest of the immunoglobulins with IgG subclasses with in normal range Neutrophil oxidative burst test,ELISA for HIV-antibody and ANA were negative. CT scan showed extensive bullous emphysematous changes, extensive consolidation and bleb formation with bronchieactatic changes of bilateral apices and right middle and lower lung fields. There was left sided pleural effusion with mediastinal lymphadenopathy CASE PRESENTATIONDISCUSSION LABORATORY FINDINGS: