Etiologies of the sicca syndrome: primary systemic amyloidosis and others.

BACKGROUND: The sicca syndrome has been defined as the occurrence of xerostomia and xerophthalmia. Sjögren's syndrome is the most common cause of the sicca syndrome; however, these two syndromes are not synonymous and there are many potential etiologies of the sicca syndrome. A less known cause of sicca syndrome is amyloidosis that to date has only been reported in the nondermatology literature. OBSERVATIONS: A 79-year-old man with known amyloidosis presented with persistent xerostomia. He had the classic cutaneous findings of periorbital and "pinch" purpura. A labial biopsy showed diffuse deposition of amorphous eosinophilic material surrounding salivary acini. Apple-green birefringence was noted with Congo red staining and the diagnosis was made of amyloidosis in the minor salivary glands causing xerostomia. CONCLUSIONS: The sicca syndrome can be caused by systemic amyloidosis. Because this fact 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 Sjögren's syndromes are important for any dermatologist.

Persistent cutaneous larva migrans due to Ancylostoma species.

Cutaneous larva migrans is considered to be a self-limited parasitic infection of about 2 to 8 weeks' duration, though it has been reported to persist for as long as 55 weeks. In this case, a healthy 47-year-old white man had multiple serpiginous lesions typical of cutaneous larva migrans for 18 months. A biopsy taken 2 months before presentation showed a parasite consistent with Ancylostoma species deep in a hair follicle. The patient initially responded to topical thiabendazole, but relapse occurred when therapy was discontinued. Oral thiabendazole cured the problem after 22 months of infestation. Cutaneous larva migrans may sometimes be long-standing, here almost 2 years, even in a healthy patient. Organisms may reside deep in the hair follicles. Topical thiabendazole may not penetrate to this depth, necessitating oral thiabendazole therapy.