HE involves many organs with predilection to skin, bone, liver and lungs.1C4 It usually manifests as multifocal nodules affecting a single organ with very low risk for metastasis to other organs.5C9 However, HE has been reported in few cases with simultaneous involvement of more than one organ.10C12 We report a 12-year-old female with EH involving lungs, NSC 405020 trachea, liver, and abdominal Tmem140 muscles who presented with nonspecific complains of weight loss, exercise intolerance and dry cough. 2.?Case Patient is a 12-year-old female patient who was referred to the respiratory department at Queen Rania Hospital in Jordan complaining of progressive exercise intolerance for the last one-year and troublesome dry cough for the last 3 months. liver, and abdominal muscles who presented with nonspecific complains of weight loss, exercise intolerance and dry cough. 2.?Case Patient is a 12-year-old female patient who was referred to the respiratory department at Queen Rania Hospital in Jordan complaining of progressive exercise intolerance for the last one-year and troublesome dry cough for the last 3 months. She lost 8?kg during her illness. NSC 405020 Chest X-ray before referral showed multiple nodules in both lungs. Based on X-ray findings, she was initially diagnosed with pulmonary tuberculosis and was treated as such with triple oral anti mycobacterial antibiotics (isoniazid, rifampicin and pyrazinamide) for two months, even though PPD test was negative. She was referred because of lack of improvement on such treatment. Patient denied any history of skin rash, joint pain, abdominal pain, abdominal distention or constipation. Rest of system review was normal. Upon examination she was frequently coughing but with no sputum production. She was in moderate respiratory distress with use of accessory muscles. Oxygen saturation was 84% on room air. Chest auscultation revealed decrease air movement on the right side with crackles heard best laterally and posteriorly. Cardiac exam revealed loud S2 sound. Abdominal examination revealed a single small nodule in the lower abdominal wall close to midline. The nodule was firm but non-tender on palpation. Liver was not enlarged and there was no splenomegaly. Skeletal muscles were wasted. Early finger clubbing was also appreciated. CBC showed moderate eosinophilia. Liver function and kidney function were normal. Serum ferritin was164?ng/ml (5-148), ESR was 12. Hb was 13.7?g/dl. Nitroblue tetrazolium was normal. Sweat chloride test was 44?Meq/L. urine analysis was normal. Carcinoempryogenic antigen, alpha fetoprotein, and Beta-HCG were normal. Antineutrophil cytoplasmic antibody NKA, antinuclear antibodies and rheumatoid factor were negative. Immunoglobulins and tissue transglutaminase were normal. 2D echocardiography showed pulmonary hypertension with mean pulmonary arterial pressure of 70?mmHg. Skeletal survey, bone isotope scan and bone marrow biopsy were all normal. Chest X-ray and chest CT scan showed multifocal nodules with ill-defined margins that were randomly distributed in both lungs with no predilection to any lobe and without cavitation (Fig.?1). Most of these lung nodules showed evidence of calcification. No mediastinal lymph node enlargement was noted. Abdominal CT scan with contrast showed multiple soft tissue attenuations in both lobes of the liver. These lesions were variable in size and with ill-defined shaggy margins and diffuse non-homogenous enhancement during the venous phase (Fig.?2). No regional or para-aortic lymph node enlargement was noted. A small mass1.5?cm in diameter was noted in the lower third of the right abdominal rectus muscle, which was strongly enhanced with contrast (Fig.?3). Open in a separate window Fig.?1 Coronal section of chest CT-scan showing multinodular lesions in both lungs. Open in a separate window Fig.?2 Abdomen CT-scan showing multinodular lesions in the NSC 405020 liver. Open in a separate window Fig.?3 CT-scan of abdomen showing enhanced lump in the right abdominal rectal muscle (arrow). Flexible bronchoscopy was performed and showed multiple small nodular lesions 1?cm below subglotic area on the right tracheal wall. Circular narrowing of the lateral segment of the middle lobe was also noted. Biopsy of the tracheal lesions showed fragments of moderately cellular proliferation of epithelioid to spindle shaped cells having large nuclei, prominent nucleoli and intracytoplasmic bubbly lumina. The cells were.