Renal disease, epidermal necrosis, and epithelial cell antibodies.
OBJECTIVE
To describe the association between epithelial cell IgM, which has previously been associated with an increased incidence of loss of renal graft in children, with a novel cutaneous eruption and unexplained native renal disease.


METHODS
Observational study on children with epithelial cell antibody presenting with unexplained renal or skin disease.


METHODS
General paediatric department and regional paediatric nephrology unit.


METHODS
Six children (five girls, one boy), who presented to the unit in 1989-90.


RESULTS
Three children, two of whom had a history of a hyperpigmented rash, presented with hypertension, proteinuria, and impaired renal function. Renal biopsy specimens from two of these children showed severe arteriolar endothelial cell swelling with arteriolar occlusion. These children fully recovered after treatment with antihypertensive drugs. The third child developed end stage renal failure and required dialysis. Three other children presented with an unusual cutaneous eruption but no evidence of renal disease. Histology of the skin lesions showed acute epidermal necrosis and features consistent with a viral infection.


CONCLUSIONS
The aetiology and pathogenesis of the epithelial cell antibody are unknown. These cases indicate that it may have a role in native kidney disease and focal epidermal necrosis. Clinical and histological features suggest that the antibody may be associated with a viral infection.
Deal
J E
JE
Department of Paediatric Nephrology, Guy's Hospital, London SE1 9RT.
Groves
R W
RW
Harmer
A W
AW
Welsh
K I
KI
MacDonald
D M
DM
Rigden
S P
SP
D058186:Acute Kidney Injury / Q000276:immunology*; D000293:Adolescent; D002648:Child; D004848:Epithelium / Q000276:immunology; D005260:Female; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology; D007075:Immunoglobulin M / Q000276:immunology*; D007668:Kidney / Q000276:immunology / Q000473:pathology; D007674:Kidney Diseases / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000276:immunology*; D008297:Male; D009336:Necrosis; D011507:Proteinuria / Q000209:etiology; D012867:Skin / Q000473:pathology*; D012880:Skin Pigmentation; D014777:Virus Diseases / Q000276:immunology
D007075:Immunoglobulin M