24 yo woman with Vasculitis

presented by

David Elpern M.D., Williamstown, MA, USA *

Lynne Goldberg, M.D., Boston, MA, USA ** and

Arash Radfar, M.D., Ph.D., Boston, MA, USA***

on January 23, 2006

* Dermatologist, The Skin Clinic, Williamstown, MA, USA

**Associate Professor of Dermatology, Department of Dermatology, Boston University, Dermatopathologist,Skin Pathology Laboratory at Boston University, Boston, MA, USA

***Assistant Professor of Dermatology, Department of Dermatology, Boston University, Dermatopathologist,Skin Pathology Laboratory at Boston University, Boston, MA, USA

Abstract 24-year-old woman with two episodes of palpable purpura over the past three years
24 yo woman
Intermittent over three years
Arms and legs

The patient is a healthy 24-year-old woman. In late October, 2005 she developed the second episode of discrete hemorrhagic papules on her legs and arms. The first episode was 3 years ago. The lesions lasted around nine months and then disappeared. They are entirely asymptomatic. The patient was seen on 12/12/05 and a diagnostic biopsy was taken. On December 27, 2005 she had a second crop of lesions, this time mostly on her arms. A second biopsy was taken of a fresh lesion.

Physical Examination

Exam shows discrete 1 - 2 mm in diameter hemorrhagic papules on proximal areas of legs and to a lesser extent on arms. There are no areas of coalescence and no dermatitic component. Post-inflammatory hyperpigmentation did not follow first episode and is not present now.


Picture taken from leg just proximal to the ankle (12/12/2005)

Laboratory Data

CBC, chemistries, ANA and other serologies all in normal range
Throat culture positive for Group A Beta Strep. (She was treated with Pen VK but was asympromatic)
Urine analysis shows microhematuria. Review of old charts show microhematuria on a number of occassions, but no other abnormalities of urine or urinary sediment and no changes in BUN or creatinine. There has never been proteinuria.

Hep Bs Antibody non-reactive
Hep C Antibody non-reactive
C-ANCA < 1:20
R-ANCA < 1:20
Complement C5 level normal


First Bx: (taken 12/12/2005)
Focal superficial dermal lymphohistiocytic infiltrate with numerous extravasated erythrocytes. NOTE : These changes are suggestive of a pigmented purpuric lichenoid dermatosis of Gougerot Blum.


2nd Bx:(taken 12/27/2005)

A subepidermal and intraepidermal vesicle containing neutrophils , overlying epidermal necrosis, a superficial perivascular and interstitial lymphocytic infiltrate containing neutrophils and rare eosinophils , and focal fibrinoid degeneration of blood vessel walls.

Diagnosis Probable Small Vessel Vasculitis
We are considering asymptomatic IgA nephropathy
Reasons Presented

Our last posting covered "Cutaneous Vasculitis." The case presented here demonstrates how difficult making an aetiologic diagnosis can be. The first biopsy did not look like vasculitis because it was from a late lesion. The patient's throat culture was positive for Group A Beta Strep. Is this related?

For opinions from our members.

To illustrate the difficulty in establishing a diagnosis of vasculitis.
To highlight how the timing of the biopsy can affect the ability to make a diagnosis.
For suggestions of whether to treat or observe.
(The patient is scheduled to see a nephrologist with regards to microscopic hematuria)

Questions Does this patient have leucocytoclastic vasculitis?
Is her microscopic hematuria in the absence of any other renal findings clinically significant?
Should her asymptomatic skin findings be treated?
How should she be followed?
Is there any value of a direct immunufluorescent skin biopsy for IgA?
Your thoughts about these and other comments will be welcome.

Coppo R, D'Amico G. Factors predicting progression of IgA nephropathies. J Nephrol. 2005 Sep-Oct;18(5):503-12. (view abstract)

Davin JC, Ten Berge IJ, Weening JJ What is the difference between IgA nephropathy and Henoch-Schonlein purpura nephritis? .Kidney Int. 2001 Mar;59(3):823-34. (view abstract)

Keywords Vasculitis, leukocytoclastic, IgA Nephropathy, Group A Beta Strep. Palpable Purpura
Comments from Faculty and Members

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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