Macroglossia is an unusual and striking clinical finding. The number of listed causes is relatively few, and the diseases associated with it are for the most part infrequently encountered. Among the listed causes are primary systemic amyloidosis, myxedema, acromegaly, glycogenosis (von Gierke's disease of muscle),1 advanced tertiary syphilis, and congenital anomaly.2 The congenital type of macroglossia is further divided into two types, lymphangiomatous and muscular. Most authors, in describing amyloidosis, have followed the classification of Reimann, Koucky, and Eklund.3 They have divided amyloidosis into four clinicopathological groups: (1) primary amyloidosis, (2) secondary amyloidosis, (3) tumor forming amyloidosis, and (4) amyloidosis associated with multiple myeloma.
Secondary amyloidosis, which is associated with various chronic diseases, is relatively common and well known, whereas primary amyloidosis is rare. Up to 1950 there had been reported in the literature approximately 70 cases. Primary amyloidosis is characterized by (1) absence of preceding disease;
Ensign WG. MACROGLOSSIA AS A MANIFESTATION OF PRIMARY SYSTEMIC AMYLOIDOSIS: REPORT OF A CASE. JAMA. 1952;149(2):136–138. doi:10.1001/jama.1952.72930190001009
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