Leprechaunism (Donohue's syndrome) . Clinical and pathologico-anatomical findings
Fürste, H.O.; Böhm, N.; Pringsheim, W.
Helvetica Paediatrica Acta 39(1): 95-104
1984
ISSN/ISBN: 0018-022X PMID: 6725000 Document Number: 221792
Leprechaunism is a very rare condition of obscure aetiology. At the age of three weeks the neonate described in this report lost all subcutaneous fat in spite of additional parenteral nutrition. He acquired purulent pneumonia, and finally died of septicaemia. The typical stigmata were a prominent nose, broad mouth with putty lips, and large, hypertrophic , backwards rotated ears, cutis laxa, atrophy of adipose tissue and gynaecomastia with hirsutism. Since the first report (1948), 32 patients have been described in detail. Compared with these reports of dysendocrinism we observed an excessive proliferation of various tissues, e.g. of the epithelia of the epidermis, bronchi, collecting tubules of kidneys, bile-ducts, and pancreatic ducts. Moreover, almost complete atrophy of lymphoid tissue was remarkable.