Chronic bilateral dacryo-adenitis in identical twins: a possible incomplete form of Sjögren syndrome.
We report an unusual case of chronic bilateral dacryo-adenitis in 10-year-old identical twin sisters. Both girls presented with bilateral lacrimal gland enlargement and developed moderate xerophthalmia and keratitis. Both the lacrimal and minor salivary gland biopsies showed a non-granulomatous inflammatory infiltration of mono-nuclear cells. All granulomatous diseases and neoplasms could therefore be ruled out and only Sjögren syndrome and very few other forms of chronic dacryo-adenitis remained as possible diagnoses. Both patients and their parents were evaluated for auto-antibodies. Very low titres of smooth muscle antibodies were found in one, antinuclear antibodies in two and anti-dsDNA antibodies in all four members of the family. Even though the titres of antinuclear and anti-dsDNA antibodies increased in one of the sisters, both patients did not develop any sign or symptom of a systemic connective tissue disease. During the 6 years' follow up, both patients showed persistent tarsal gland enlargement but no other symptoms apart from a moderate xerophthalmia and occasional mild keratitis.
Besana
C
C
Department of Internal Medicine, Università di Milano.
Salmaggi
C
C
Pellegrino
C
C
Pierro
L
L
Vergani
S
S
Faravelli
A
A
Rugarli
C
C
D001706:Biopsy; D002648:Child; D002908:Chronic Disease; D003607:Dacryocystitis / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D004200:Diseases in Twins; D005260:Female; D006801:Humans; D007634:Keratitis / Q000150:complications / Q000235:genetics; D012470:Salivary Glands, Minor / Q000473:pathology; D012859:Sjogren's Syndrome / Q000235:genetics*; D014430:Twins, Monozygotic; D014985:Xerophthalmia / Q000150:complications / Q000235:genetics