Rick Sontheimer MD, Professor and Vice-Chairman,
Dept. of Dermatology University of Oklahoma Health Sciences
Center, Oklahama City,OK, USA on Jan 23, 2006
Her young age, the recurrent crops of purpuric macukes/papules
especially involving the upper extremities, the recent biopsy
showing leukocytoclastic vasculitis, and the microscopic hematuria
to me are suggestive of Henoch-Shoenlein purpura (HSP) with
renal involvement.
Yes, I think that a direct immunofluorescence exam of a lesional
skin biopsy showing IgA-predominant microvascular immune deposits
would confirm the diagnosis of HSP. Unless symptomatic, her
skin lesions themselves do not necesdsarily require treatment.
However, her skin lesion activity might be used as a surrogate
marker for her systemic HSP involvement (renal). Renal consultation
should guide approach to follow-up.
If her direct immunofluorescence examination did not reveal
an IgA-predominant vasculitis, I would then order a Ro/SS-A
autoantibody since this is one of the few lupus- and Sjogren's
syndrome-related autoantibodies that can be positive when an
ANA is negative.
Kenneth Becker M.D. Providence, RI, USA on
Jan 23, 2006
I think this patient has HSP.
Tan, Heng Soon MD, Brigham and Women's Primary Care
Associates at Newton Corner, Harvard Medical School, Boston,
MA, USA on Jan 24, 2006
Clinically she has Henoch Schonlein purpura that is frequently
associated with asymptomatic microhematuria. Diagnosis is made
on skin biopsy that shows small vessel vasculitis consistent
with leucocytoclastic vasculitis. IgA deposits can be found
in both skin and renal biopsy. Though vasculitis may be recurrent,
no treatment is necessary since the prognosis is good with spontaneous
recovery and no long term sequelae.
IgA nephropathy is at risk for progression only in presence
of increasing proteinuria and later development of impaired
renal function and hypertension. The presence of asymptomatic
strep pharyngitis raises the possibility of poststrep glomerulonephritis
to explain microhematuria, however, this is not consistent with
recurrent vasculitis. So her clinical presentation is best explained
by Henoch Schonlein purpura.
I enclose relevant paragraphs from Up To Date and PubMed that
provide more discussion:
( see details )
Andrew Carlson MD, Professor,
Divisions of Dermatopathology and Dermatology, Albany Medical
College, Albany, NY, USA on Jan 24, 2006
1) Does this patient have leucocytoclastic vasculitis?
Yes, the biopsy clearly shows requiste features for a diagnosis
of leukocytoclastic vasculitis (vascular fibrin deposits, perivascular
neutrophilic debris and disruptive neutrophilic infiltrate associated
with extravasated red blood cells). The fibrosis, albeit slight,
is suggestive of an early lesion of erythema elevatum diutinum.
The wedge pattern of fibroinflammatory changes raises the possibility
of preceding folliculitis or so-called sterile neutrophilic
folliculitis with perifollicular vasculopathy as described by
Magro which is associated with infections such as strep pharyngitis.
2) Is her microscopic hematuria in the absence of any other
renal findings clinically significant?
Yes, it's an indication of renal vasculitis, and likely possibility
of Henoch-Schonlein purpura.
3) Should her asymptomatic skin findings be treated?
How should she be followed?
Supportive care and investigation for trigger or underlying
disease. In this case, it's likely infectious based on positive
strep swab of throat.
Long-term follow-up is important for children and adults with
Henoch-Schönlein purpura because renal impairment can be
seen many years after the initial diagnosis. Consideration of
a renal biopsy is believed to be necessary by some authorities
to define long-term prognosis for these patients. In addition,
there exists a specific population at high risk of a poor outcome
based on clinical and histological assessments- she doesn't
fit the findings described for this population. See below.
Kawasaki, Y., J. Suzuki, et al. (2003). "Clinical and
pathological features of children with Henoch-Schoenlein purpura
nephritis: risk factors associated with poor prognosis."
Clin Nephrol 60(3): 153-60. (view
abstract)
4) Is there any value of a direct immunufluorescent skin biopsy
for IgA?
Yes, one cannot confirm the diagnosis of HSP without documentation
of IgA vascular immune complexes.
Janjua Shahbaz MD, Specialist Dermatologist, Ayza Skin
and Research Center, Lalamusa, Pakistan on Feb 5, 2006
Cutaneous eruption, evidence of renal involvement(although
subtle), and a raised ASO titre are suggestive of HSP in this
case. No treatment is necessary for the cutaneous symptoms.
I would prefer to check serum IgA levels than a direct immunoflourescence
to confirm the diagnosis.
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