Purpuric Vasculitis

Chronic relapsing, non progressing eruption of purpuric papulovesicular lesions
Short Summary

A 57 year old female complained about appearance of red to dark red pruritic bullous lesions . These lesions were, and still are, located on the legs from the knee to the ankles, and on the back of the feet. These manifestations appeared in variable phases during the next 4 years, with episodes of more numerous pustules appearing in some periods, and more isolated in other periods.

The patient underwent specialist dermatological examinations and haematic analyses particularly for complete blood count and autoantibody research , the reports of which are attached below .Viral genome Parvovirus B19 research was negative. The medical tests carried out  led to a diagnosis of “Purpuric Vasculitis”, with findings of microhaematuria and hypothyroidism.

The treatment carried out  was based on  steroids and Vitamin C. 

Patient's Questions

1) What do you think is the cause of the disease?
2) Is there a treatment that leads to final recovery?
3) What are the consequences, if any, of this disease?

 

Medical Background

57 years old, female.

Symptoms arose in September 2006 with the appearance of red to dark red pruritic bullous lesions of a circumference generally equal to that of a lentil, with some even larger. These lesions were, and still are, located on the legs from the knee to the ankles, and on the back of the feet.
In the beginning the patient attributed these manifestations to insect bites, so they were treated with antihistamines both orally and locally.


It was only in January 2007 that the patient underwent specialist dermatological examinations and was prescribed pharmacological treatments (local cortisones and antihistamines taken orally), occlusive bandages, haematic analyses particularly for complete blood count, autoantibody research (ANA,  ASMA,Anti TPO, Anti-Jo-1, ASA, ACA,  IgG, IgM and IgA), the reports of which are attached below. Viral genome Parvovirus B19 research was also carried out, which was negative, as wells as a histopathological examination of left perimalleolar skin with the report coming back “livedo, eczematized vasculitis".


Reassessed by the dermatologis on February 20, 2007, a new treatment was prescribed that included Vitamin C, 1-g vial to drink at the dosage of 1 per day for 2 months, + Diraisth cap at the dosage of 3 caps per day for 10 days, then 2 caps per day for another 10 days, and finally 1 cap per day for another 10 days, + Nerisona cream at the dosage of 3 applications per day + Zirtec cap at the dosage of 1 cap per day for 20 days.
The result of these treatments was that there was considerable improvement of the symptoms, almost to the point that they totally disappeared.

 

However, About two months later the bullous lesions progressively spreaded until they became particularly intense and pruritic, so much so as to necessitate repetition of the immunologic analyses (annexes 7, 8, 12 and 13), the dermatological examinations and resumption of the cortisone treatment.
On this occasion as well the symptoms improved but only in part and temporarily, thus forcing the patient to be hospitalized .


The medical tests carried out during hospitalization led to a discharge diagnosis of “Purpuric Vasculitis” with finding of microhaematuria. It is also reported that hypothyroidism was diagnosed during hospitalization. The treatment carried out during hospitalization was based on daily infusions of steroids and Vitamin C. Immunologic exams and a cutaneous biopsy were also performed, the report of the relevant histological exam of which is provided: “section of skin with dermal vessels dilated, focal and modest fibrinoid degeneration of the walls of some small superficial vessels, extravasation of erythrocytes and superficial dermal infiltrate of eosinophilic lymphocytes, histiocytes and granulocytes". Neither was the improvement following the treatment prescribed during hospitalization definitive, so a few months later the disease resumed its course, although in a less violent form.


In short, the manifestations continued in variable phases during 2008 and 2009, with episodes of more numerous pustules appearing in some periods, and more isolated in other periods. The oral cortisone treatments that the patient followed during these two years was suspended both upon the advice of the family doctor and due to the onset of collateral effects (weight increase, down, etc.).

 

Laboratory exams:

Complete blood count-

  Hemoglobin-14.2 gr/dl

  Hematocrit- 42.5%

  RBC- 4.79 *106/µl

  MCH- 29.6 pg

  MCV- 88.7 fL

  MCHC- 33.4 g/dl

  RDW- 11.8%

  Plateletes- 364

  WBC- 10 *103/µl

  Neutrophils- 55%

  Eosinophils- 3%

  Lymphocytes- 37%

  Monocytes- 5%

 

Autoantibody research-

  ANA -anti nuclear antibodies(SSA,SSB,SM,RNP)- <25 u/ml.

  Anti TPO antibodies- 41 u/ml.

  Anti thyroglobulins antibodies- 38 u/ml.

  ASMA (anti smooth muscle antibodies)- negative.

  Anti-Jo-1- <25 u/ml.

  ASA (anti sarcomere antibodies)- negative.

  ACA (anti centomere antibody)- negative.  

 

Expert's Opinion

This is an opinion based on the attached clinical information, including lab reports, and a close-up photo of one of her lesions. I have not performed a clinical examination of the patient. Information on her past history, including drug intake, is lacking.


This is a 57 y/o woman, who has been suffering from a chronic relapsing, non progressing eruption of 4 years' duration, associated with marked itch. The eruption is manifested by purpuric papulovesicular lesions located over the skin of her both legs and dorsal aspects of the feet. On the basis of the clinical and histopatholgical findings the patient was diagnosed as having purpuric vasculitis.
Overall, the course of the eruption, the itch , the morphology of the lesion (as it is shown in the clinical photo provided) are all not characteristic to any of the long list of small vessel vasculitis. Histopathological examination of the first biopsy specimen was interpreted as "livedoid, eczematized vasculitis" without any further detailed description. However, the clinical features are not characteristic to livedoid vasculitis/vasculopathy. The second biopsy showed focal and modest fibrinoid degeneration of the superficial dermal blood vessels, extravasation of erythrocytes and polymorphic superficial infiltrate including eosinophils and neutrophils . These findings are not diagnostic to livedoid vasculitis, mainly due to the accompanied dermal infiltrate. Neither is it diagnostic to leukocytoclastic vasculitis, since nuclear fragmentation of the neutrophils was not mentioned in the histopathological report.


An extensive laboratory investigation, including sedimentation rate hematogram, autoantibodies, serology for infectious agents- was all normal or negative. Urinalysis was also normal.

On the basis of the available data, the differential diagnosis of this eruption includes:


1. Unusual vasculitis/ vasculopathy
In the light of the histopathological findings, the following additional investigations are suggested: direct immunofluorescence (DIF), anti cardiolipin – IgA (IgG, IgM- were normal) and beta 2 glycoprotein for IgG, IgM, IgA isotypes, rheumatic factor, cyroglobulines, anti hepatitis B and C antibodies.
Due to drug eruption? Paraneoplastic eruption? Given the protracted course this possibility is less likely.

Nevertheless, I would order radiologic imaging. Also I did not see the blood levels of LDH.


2. Unusual localized form of bullous pemphigoid

I would suggest to obtain an additional biopsy from a bullous lesion, since bulla formation was not mentioned in any of the histopathological reports. Also, a biopsy from a perilesional skin for DIF is mandatory.

It is difficult to recommend a treatment in the absence of a diagnosis.
However, given the presence of neutrophils and eosinophils, dapsone or colchicine are reasonable steroid sparing options for her eruption.

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