Abstract:
This report describes a rare case of Juvenile Systemic Lupus Erythematosus (JSLE). A young 13-year-old girl presented to the Civil Hospital Karachi on February 15, 2019 with gangrene as the only manifestation of this autoimmune disease. JSLE has several clinical manifestations such as butterfly rash, fever, joint pain, cardiac problems like pericardial infusion and neuropsychiatric disorders. However, in this case gangrene was the only presenting symptom; only laboratory investigations - anti-SSA and antiribosomal P protein - were suggestive of JSLE, while anti dsDNA, considered to be the most sensitive and reliable diagnostic tool for Systemic Lupus Erythematosus (SLE), was negative. Raynaud's phenomenon and gangrene have been described as rare symptoms, with gangrene occurring in only a small percentage of SLE patients. Moreover, the patient had received a blood transfusion a few months ago in Hyderabad which was suspected to be the cause of the transmission of infection which lead to polyclonal activation of lymphocytes.
Page(s):
1869-1873
DOI:
DOI not available
Published:
Journal: Journal of Pakistan Medical Association, Volume: 70, Issue: 10, Year: 2020
Keywords:
Vasculitis
,
Autoimmune
,
Juvenile Systemic Lupus Erythematosus
,
Gangrene