A 16-year-old previously healthy female presented to this hospital with a two-year history of treatment-refractory tinea capitus of the scalp, left ear, and neck. At that time, she denied fever, malaise, arthralgia, fatigue, weight loss, or any other constitutional symptoms. She further denied cough, shortness of breath, or dyspnea. Her tinea capitus ultimately was treated successfully by terbinafine after failing several other antifungal regimens.
One year later, the patient presented again to this hospital with an eight-month history of chronic cough with decreased energy, a 15-pound weight loss, and cervical lymphadenopathy. During the three months before admission, she developed a right-sided neck mass that had been nontender and not inflamed. She was afebrile. She had been seen by her primary care physician and subsequently referred for a biopsy of the neck mass about two weeks before admission. A chest radiograph showed diffuse, widespread pulmonary disease with associated lymphadenopathy. Pathologic analysis of the cervical lymph node biopsy demonstrated findings consistent with Coccidioides infection. Serology also revealed a coccidioidal antigen antibody titer of 1:128 (no antibodies are detected in normal individuals). The patient had a negative Purified Protein Derivative (PPD) tuberculosis test.
CT of the abdomen and pelvis without contrast revealed bilateral pleural effusions and multiple small pulmonary nodules seen throughout all pulmonary lobes. Cavitation was seen in a right lower lobe consolidation, with the formation of a bronchopleural fistula. Moderate compression of the superior vena cava and right pulmonary artery were noted, but no intraluminal filling defects were observed. An enlarged and presumably infected right inferior sternocleidomastoid muscle was also noted. Additionally, the patient had significant hepatosplenomegaly with diffuse calcifications dispersed throughout the liver and spleen. A periportal mass was also observed. The kidneys bilaterally had multiple calcified lesions, and a necrotic node was noted in the peritoneum. Additionally, there were multiple retroperitoneal and mesenteric node calcifications. Free fluid was seen in the right pericolic gutter, within the pelvis and bilateral lower quadrants. Multiple lytic lesions were visualized within the right clavicle and right aspect of the T6 vertebral body, the right T5 vertebral body, the sternum, and the right transverse process of the T10 vertebra. Spina bifida was observed at S1. The bowel appeared normal in caliber and free of involvement. The pancreas, adrenal glands, gallbladder, and bladder also appeared unremarkable.
CT of the head further revealed incidental bilateral basal ganglia calcifications, but was otherwise unremarkable. There was no definite acute intracranial hemorrhage or territorial infarct. There was no mass effect or hydrocephalus. The orbits and paranasal sinuses were within normal limits. No suspicious focal osseous lesions were present. CT of the sinuses and orbits revealed mucous retention cysts involving the left maxillary and right sphenoid sinuses, without air-fluid levels to suggest acute infection. In the context of this patient's presentation with chronic cough, immune deficiency, negative PPD, and residence in southern Arizona, the imaging findings presented here are most consistent with disseminated coccidioidomycosis infection.
Since this admission, the patient's condition has improved considerably. The patient initially required a gastric tube for feeds, but has since had the tube removed and is now feeding orally and maintaining a normal body weight. The patient is currently maintained on prophylactic Posaconazole (a triazole with more efficacy than fluconazole or itraconazole in the prevention of fungal infections) and trimethoprim-sulfamethoxazole for her immunodeficiency. Followup imaging has not been conducted. The exact cause of the patient's immunodeficiency remains undetermined. Several laboratory results for specific known causes of immunodeficiency returned negative or unequivocal during her hospital stay. At the time of publication, the Infectious Disease group was following the case closely and preparing to send the patent to Bethesda, Maryland to the National Institutes of Health for further genetic testing.