Journal of Clinical & Experimental Pathology
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Erdheim-Chester Disease (ECD), a rare multisystemic non-Langerhansâ???? cell histocytosis of unknown etiology, primarily
affects middle-aged adults. Pulmonary manifestations occur in 15-33% of patients and significantly increase morbidity
and mortality. We report a 60-year-old female smoker who presented with dyspnea, weakness and recurring reticulonodular
lung infiltrates on CT imaging. Past medical history includes lower extremity pain, bilateral hip replacement and shoulder
arthroplasty for degenerative joint disease. Chest radiographic findings were suggestive of possible malignancy or sarcoidosis
with progressive centrilobular nodules in a tree-in-bud pattern. Bronchoscopic examination was negative for obstructive
endobronchial lesions. The histologic findings on right lung wedge resection revealed interstitial infiltration by finely to
coarsely vacuolated macrophages, occasional lymphoid aggregates, and collagenous fibrosis in a perivascular, interlobular
septal, and peribronchial pattern. Few macrophages were present in alveolar spaces possibly suggesting chronic exogenous
lipoid pneumonia (Fig. 1A and B). An isolated necrotizing granuloma of presumed infectious etiology was also observed.
A six-year interval progression of her interstitial lung disease prompted repeat surgical biopsy, of the left lung, with similar
histological findings, excluding necrotizing granulomas. Immunophenotypically, the histiocytes were CD68 positive, focally
positive for Factor XIIIa and negative for S100 and CD1a (Fig. 1C). Review of the slides of left shoulder bone and synovium
showed dense infiltration of histologically and immunophenotypically similar histiocytic cells (Fig. 1D), supporting the
diagnosis of ECD. This case raises the question of possible predisposition to infectious granulomatous disease in ECD and
emphasizes the importance of review of prior non-pulmonary biopsies to establish the diagnosis, especially in radiographically
or histologically atypical cases.
Karam Han has completed her medical degree from Boston University School of Medicine. She is currently completing her residency at MetroHealth Medical Center, Case Western Reserve University in Cleveland, Ohio.