Teaching Case

Patient presented with fever (38ºC) and productive cough a month ago.

The patient has a history of several hospital admissions since early childhood, due to reccurent pulmonary infections as well as sinusitis, treated surgicaly twice. Repeated thoroughly investigations in several clinics since the age of 28, have failed to show the existence of an underlying systemic disease. A CT examination revealed severe destructive bronchitis, early bronchiectatic changes of the left lower lobe and lingula. Most of her pulmonary infections were due to common bacteria, except in one admission were Aspergillus was isolated. At the age of 31 she developed extensive ulcerative lesions of the face and buccal mucosa, which have been proved to be non-specific granulomas under biopsy. Liver biopsy revealed non-specific granulomatous hepatitis. Cultures from face and sputum isolated Candida. Thorough immunologic investigation indicated that neutrophil function was entirely normal while T-cell subset in response to delayed hypersensitivity had some problems with very low titer to Candida. Thus it was concluded that the patient suffered from a rare syndrome of chronic mucocutaneous candidiasis. She underwent a life-long treatment with fluconazole which had excellent results on skin lesions. Prophylactic therapy with trimethoprim-sulfamethoxazole was also applied for bronchiectasis.

Chronic mucocutaneous candidiasis is a rare complex syndrome characterized by persistent and recurrent Candida albicans infection of mucous membranes, skin and nails. There are several subgroups of patients with chronic mucocutaneous candidiasis and these can be identified by associated disorders such as immunodeficiency (cellular or humoral deficiency, partial or complete), autoimmune diseases, endocrinopathies, thymoma (1). Children suffering from chronic mucocutaneous candidiasis also presented with a remarkable susceptibility to non candidal infections, with reccurent bacterial pneumonias and bronchiectasis being the major cause of morbidity and mortality (2). Interpretation of hepatic granulomata is problematic since they occur not only in patients with systemic granulomatous disease, but also in a variable number of patients with undrlying liver disease and in a heterogenous group of patients with disorders that appear to be of neither hepatic nor granulomatous in nature (3). Bronchiectasis is associated with a variety of predisposing conditions and no specific correlation to Candida infection (4). Bronchiectatic changes were identified with HRCT (the imaging modality of choice for demonstrating or ruling out bronchiectasis and its extent) (5) after repeated pulmonary infections in early adulthood. Several immunodeficiency states are currently investigated in early childhood for patients presenting with reccurent pulmonary infections. In our patient there was not detected any primary immunodeficiency disease (6). We provide an illustrative case report and discussion on a patient suffering from diffuse bronchiectatic changes since early childhood with an underlying disease of chronic mucocutaneous candidiasis and concomitant reccurent pulmonary infections. Regarding the imaging technique, 3mm collimation scans was obtained at 3mm intervals through the chest with a window level of -700 HU anda a width of 1000 HU. Bronchiectatic changes are seen widespread in both lungs, but more pronounced at the lower lobes. There is failure of the bronchi to taper towards the periphery and are often visualized in close proximity to the costal pleura. Increased bronchial wall thickness is apparent. The morphology of bronchiectatic changes are mainly cylindrical with signet-ring and "tram line" configuration. There are small centrilobular nodules widespread that represent thickening of the bronchial wall or filling of dilated bronchioles with granulation tissue, mucus or pus. There was not detected pleural or pericardial fluid nor pathological enlarged mediastinal lymph nodes.