대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Chronic Mucocutaneous Candidiasis with Recurrent Bronchopneumonia and Calcification of Thoracic Aorta

Radiologic Findings

Chest radiograph shows multiple areas of small nodular and ground-glass opacities in the right upper and left lower lung zone. Note linear calcifications along the course of ascending thoracic aorta. Precontrast CT scan shows diffuse calcification in thoracic aorta. Chest CT scans with lung window setting show ground glass and multiple small nodular opacities in right upper lobe and left lower lobe. Many of them are centrilobular in location. Also note tubular bronchiectasis in left lower lobe.

Endoscopy showed multifocal whitish small plaques in almost whole esophageal wall and mucosal ulceration in the mid esophagus. Barium esophagography revealed a deep ulcer in the mid esophagus (not shown).
The patient had long standing history of mucocutanous candidiasis and recurrent respiratory infection from birth. Bronchoalveolar lavage revealed streptococci and cryptococcus. Although cryptococcus might be ascribed to contamination, his respiratory symptoms and radiographic findings improved with antifungal and antibacterial treatment. He was not thought to have any endocrine dysfunction and endocrinological evaluation was not performed.

Brief Review

Chronic mucocutaneous candidiasis (CMC) is an uncommon disease characterized by refractory candida infection of mucus membranes, nail and skin, with onset in childhood. Some of the conditions have genetic predispositions. A defect of T cell mediated cellular immunity against candida albicans is a consistent finding: many of the patients have other immune system abnormalities resulting in endocrine dysfunction and coexisting non-candida infections. In contrast to the patients with secondary immunosuppressive conditions (such as observed in malignancy patients and their treatment complications) systemic candidiasis rarely occurs in CMC (1, 2). However, severe non-candida infections can occur including disseminated histoplasmosis. Pulmonary cryptococcosis also can occur. Bacterial infections have included s. aureus pneumonia and sepsis. The autoimmune disorders have included neutropenia, thrombocytopenia, anemia, uveitis and juvenile arthritis (2). Some of the patients were reported to have endocrinopathies including thyroid, parathyroid, and adrenal dysfunction). Slowly progressive vascular calcification in the patients with CMC even in very young age, although pathogenesis is unexplained to date, appears to be a non-random part of the more common candidiasis-endocrinopathy syndrome (2).

References

1. What syndrome is this? Chronic mucocutaneous candidiasis. Pediatr Dermatol. 2001 Jul-Aug;18(4):353-5.
2. Thoracic aortic calcification in 3 children with candidiasis-endocrinopathy syndrome. Pediatr Radiol. 1993;23(2):100-3.

Keywords

Lung, Vascular, Infection, immune related,