Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia.
OBJECTIVE
To report three cases of pulmonary or myocardial disease (or both) and necrobiotic xanthogranuloma.


METHODS
Giant cell granulomas of the lung and myocardium were demonstrated in three patients who had pulmonary and myocardial lesions of necrobiotic xanthogranuloma in conjunction with skin lesions, leukopenia, paraproteinemia, and complement deficiencies. The patients were two men who were 47 and 64 years of age and a 39-year-old woman.


RESULTS
Biopsies of skin and visceral lesions showed asteroid and cytoplasmic inclusions. B-cell lymphoid nodules were found. In one of the male patients, a major clonal T-cell receptor gene rearrangement was detected in the peripheral blood. Prednisone was ineffective in two of the patients. The other patient experienced regression of skin lesions and diminishment of a chest nodule after receiving alkylating agent therapy.


CONCLUSIONS
Establishing the correct diagnosis is important, and apparently it is possible to establish the nature of the myocardial and pulmonary lesions with use of appropriate scans and by biopsy. Successful treatment of necrobiotic xanthogranuloma skin lesions with corticosteroids or alkylating agents (or both) implies that evolution of serious disease that compromises the heart and lungs could be controlled.
Winkelmann
R K
RK
Department of Dermatology, Mayo Clinic Scottsdale, Arizona, USA.
Litzow
M R
MR
Umbert
I J
IJ
Lie
J T
JT
D000328:Adult; D001344:Autopsy; D001706:Biopsy; D009202:Cardiomyopathies / Q000150:complications / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006101:Granuloma, Giant Cell / Q000150:complications / Q000473:pathology*; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009335:Necrobiosis Lipoidica / Q000150:complications / Q000473:pathology*; D010265:Paraproteinemias / Q000150:complications* / Q000473:pathology; D012867:Skin / Q000473:pathology*; D014973:Xanthomatosis / Q000150:complications / Q000473:pathology*