Dados do Trabalho

Title

SHOENFELD'S SYNDROME AFTER LOCAL VITAMINS A, D AND E COMPLEX INJECTIONS: A CASE REPORT

Background

The autoimmune/inflammatory syndrome induced by adjuvants (ASIA) or Shoenfeld's syndrome is a recently identified condition in which the exposure to certain substances, such as silicone, vaccine adjuvants, and squalene, leads to an aberrant autoimmune response. Injection of mineral oils have also been implicated in the development of the syndrome, with formation of sclerosing lipogranuloma. Here, we present a rare case of veterinary vitamins A, D and E (ADE), an oil complex, inducing ASIA.

Case report

A 34-year-old female patient was admitted to our service to investigate skin thickening in the thighs, which biopsy showed scleroderma compatible with fibrosing dermatites. She also had calcinosis in the abdomen, buttocks and thighs, and diffuse facial erythema, with positive anti-nuclear antibody test (1:640 nuclear speckled pattern), transiently positive lupus anticoagulant, and proteinúria of 2,9g/day . Other autoantibodies, such as anti-dsDNA, anti-Sm, anti-SSa, and anti-RNP, were negative. Renal biopsy was performed, evidencing global and segmental glomerulosclerosis with deposits of C3c and fibrinogen on direct immunofluorescence. Patient reported that, eight years earlier, she self-injected ADE in the region of the cutaneous lesions with the purpose of cosmetic muscular definition.

Conclusion

The authors believe that previous exposure to ADE is related to ASIA in the present case. This association is very rare, but it has been reported before and it should be remembered as a differential diagnosis of scleroderma, specially in atypical cases. The relationship between adjuvants and the imune system is still unclear and it requires further investigation in order to better understand the pathophysiology and to indetify associated risk factors predisposing to the condition.

Área

Rare Diseases