D. Belder-Preston et al. / Open Journal of Pediatrics 1 (2011) 67-71
70
the hyperinflammatory state, enabled mounting of a
pathologic immune response with production of autoan-
tibodies. Upon removal of the antigenic stimulation, the
pathologic production of autoantibodies ceased, leading
to their disappearance from the circulation. This hy-
pothesis is supported by the fact that, 23 months after
initial presentation, the patient remains off any therapy
and healthy.
Cotton wool spots, which were ob ser v ed in our patien t,
often indicate a serious systemic disease. They represent
acute, focal, inner retinal ischemia and may occur in any
disease that compromises arteriolar circulation to the
inner retinal layers which includes SLE and systemic
vasculitides where ischemia is caused by thrombogenic
effects from antigen-antibody complexes [20]. However,
similar to our patient, isolated, self-limited cotton wool
spots in patients with no identifiable systemic disease
have also been reported [21].
The presence of pericardial effusion in our patient
could indicate SLE as pericarditis is the most common
cardiac manifestation of this disease. However, transient
asymptomatic pericardial effusions are often seen in
instances of significant systemic inflammation, espe-
cially in the presence of accompanying hypoalbumine-
mia [22]. Our patient did have hypoalbuminemia (data
not shown). The pericardial effusion in our patient was
asymptomatic with spontaneous resolution. Pericarditis
accompanying KFD has not been previously described.
Histologically, SLE and KFD can be very challenging
to differentiate and at times impossible. In KFD, histo-
pathological findings include varying degrees of necrosis,
histiocytic proliferation with activated T lymphocytes,
small lymphocytes and plasma cells without granuloma-
tous inflammation with absent neutrophils and eosino-
phils. Adjacent vessels may be thrombosed. In contrast
to KFD, SLE lymphadenitis, demonstrates hematoxylin
bodies (aggregates of degenerated nuclear debris) and
Azzopardi phenomenon (degenerated nuclear material
aggregated in the walls of blood vessels). In SLE, abun-
dant plasma cells, prominent reactive follicular hyper-
plasia, sparse cytoxic T cells and capsular and pericap-
sular inflammation are seen [23,24]. In our patient’s bi-
opsy specimens, the presence of extensive necrosis
without neutrophilic inflammation or granulomas and
the absence of hematoxylin bodies support the diagnosis
of KFD rather than SLE or systemic vasculitis.
In conclusion, this report discusses a case of atypical
KFD and illustrates several overlapping features of KFD
and SLE. Our patient presented with retroperitoneal
lymphadenopathy, an unusual manifestation of KFD. He
demonstrated a transient elevation of autoantibodies
typically associated with SLE, as well as retinal vascu-
litis and pericardial effusion, not previously described in
patients with KFD. This illustrates the need for careful
differential diagnosis, as KFD is self-limited and, con-
trary to SLE, does not require long-term immunosup-
pressive therapy; ra ther, often only supportive ther apy is
needed.
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