Digital clubbing in primary intestinal lymphangiectasia: a case report.
Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.
Wiedermann
Christian J
CJ
Division of Internal Medicine, Central Hospital, Bolzano, Italy. christian.wiedermann@asbz.it
Kob
Michael
M
Benvenuti
Stefano
S
Carella
Rodolfo
R
Lucchin
Lucio
L
Piazzi
Lucia
L
Chilovi
Fausto
F
Mazzoleni
Guido
G
D001706:Biopsy; D003131:Combined Modality Therapy; D018752:Diet, Fat-Restricted; D058582:Double-Balloon Enteroscopy; D004750:Enteral Nutrition; D005526:Food, Formulated; D006801:Humans; D007421:Intestine, Small / Q000473:pathology; D008201:Lymphangiectasis, Intestinal / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology; D008209:Lymphedema / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010005:Osteoarthropathy, Secondary Hypertrophic / Q000178:diet therapy / Q000209:etiology*; D011504:Protein-Losing Enteropathies / Q000175:diagnosis / Q000178:diet therapy / Q000473:pathology
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