Author/Editor		Ferluga, D; Hvala, A; Kuhar, M; Accetto, M; Vizjak, A
Title		Peculiar hyalinizing lupus microangiopathy in childhood mimicking clinically hypocomplementemic urticarial vasculitis
Type		članek
Source		In: Vizjak A, Rozman B, Ferluga D, editors. Autoimmune systemic diseases. Proceedings of the 30th memorial meeting for professor Janez Plečnik; 1999 Dec 2-3; Ljubljana. Ljubljana: Faculty of medicine, Institute of pathology,
Publication year		1999
Volume		str. 119-22
Language		eng
Abstract		Diagnostic criteria for primary hypocomplementemic urticarial vasculitis syndrome (HUVS) have been proposed. Nevertheless, clinical similarities and overlapping with other immune complex systemic diseases, particularly systemic lupus erythematosus (SLE), have to be considered. Using traditional light, immunofluorescence and electron microscopic techniques, skin and kidney biopsies were studied in four children patients fulfilling basic and more than two minor criteria for HUVS. During a clinicat follow-up diagnostically exclusive high level of anti-DNA in the serum was demonstrated in two patients. Histopathological study provided further evidence against HUVS and suggested that our cases represent a peculiar subset of SLE: severe progressing mixed glomerulonephritis in a 8-year-old girl, full-house glomerular, tubulo-interstitial and vascular immune deposits, fingerprint-like deposits in femak sibling and a 8-year-old girl and in all four patients an unique finding of immune complex hyalinizing small arteriolitis and sharing similarities with characteristic lupus microangiopathy.
Descriptors		VASCULITIS, ALLERGIC CUTANEOUS LUPUS ERYTHEMATOSUS, SYSTEMIC FLUORESCENT ANTIBODY TECHNIQUE MICROSCOPY, ELECTRON GLOMERULONEPHRITIS URTICARIA CRYOGLOBULINS ANTIBODIES, ANTINUCLEAR