ETIOLOGIES OF THE SICCA SYNDROME - PRIMARY SYSTEMIC AMLOIDOSIS AND OTHERS

Background. The sicca syndrome has been defined as the occurrence of x erostomia and xerophthalmia. Sjogren's syndrome is the most common cau se of the sicca syndrome; however, these two syndromes are not synonym ous and there are many potential etiologies of the sicca syndrome. A l ess known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature. Observations. A 79-ye ar-old man with known amyloidosis presented with persistent xerostomia . He had the classic cutaneous findings of periorbital and ''pinch'' p urpura. A labial biopsy showed diffuse deposition of amorphous eosinop hilic material surrounding salivary acini. Apple-green birefringence w as noted with Congo red staining and the diagnosis was made of amyloid osis in the minor salivary glands causing xerostomia. Conclusions. The sicca syndrome can be caused by systemic amyloidosis. Because this fa ct is not in the dermatologic literature, many dermatologists are not aware of this uncommon presentation. The knowledge of the many causes of the sicca syndrome and an understanding of the differences between this and Sjogren's syndromes are important for any dermatologist.